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1. Influence of invasive aspergillosis during acute leukaemia treatment on survival after allogeneic stem cell transplantation: a prospective study of the EBMT Infectious Diseases Working PartyResearch in context

Author

Olaf Penack, Gloria Tridello, Urpu Salmenniemi, Rodrigo Martino, Nina Khanna, Katia Perruccio, Franca Fagioli, Monika Richert-Przygonska, Hélène Labussière-Wallet, Johan Maertens, Charlotte Jubert, Mahmoud Aljurf, Herbert Pichler, Gergely Kriván, Desiree Kunadt, Marina Popova, Melissa Gabriel, Elisabetta Calore, Igor Wolfgang Blau, Fabio Benedetti, Maija Itäla-Remes, Elizabeth de Kort, Domenico Russo, Maura Faraci, Anne-Lise Ménard, Peter von dem Borne, Xavier Poiré, Akif Yesilipek, Jolanta Gozdzik, Zeynep Arzu Yeğin, Lucrecia Yañez, Luca Facchini, Gwendolyn Van Gorkom, Lorenz Thurner, Ulker Kocak, Antònia Sampol, Tsila Zuckerman, Marc Bierings, Stephan Mielke, Fabio Ciceri, Lotus Wendel, Nina Knelange, Malgorzata Mikulska, Dina Averbuch, Jan Styczynski, Rafael de la Camara, and Simone Cesaro

Subjects
Invasive, Aspergillosis, Stem cell transplantation, Mortality, Medicine (General), R5-920
Abstract

Summary: Background: Infections are the main reason for mortality during acute leukaemia treatment and invasive aspergillosis (IA) is a major concern. Allogeneic stem cell transplantation (alloSCT) is a standard therapy and often is the only live-saving procedure in leukaemia patients. The profound immunodeficiency occurring after alloSCT led to high IA-associated mortality in the past. Therefore, patients with IA were historically considered transplant-ineligible. Recently, there has been improvement of anti-fungal management including novel anti-fungal agents. As a result, more leukaemia patients with IA are undergoing alloSCT. Outcome has not been prospectively assessed. Methods: We performed a prospective study in acute leukaemia patients undergoing alloSCT to analyse the impact of a prior history of probable or proven IA (pre-SCT IA). The primary endpoint was 1-year non-relapse mortality (NRM). Relapse free survival and overall survival were analysed as secondary endpoints. Findings: 1439 patients were included between 2016 and 2021. The incidence of probable or proven pre-SCT IA was 6.0% (n = 87). The cumulative incidence of 1-year NRM was 17.3% (95% CI 10.2–26.0) and 11.2% (9.6–13.0) for patients with and without pre-SCT IA. In multivariate analyses the hazard ratio (HR) for 1-year NRM was 2.1 (1.2–3.6; p = 0.009) for patients with pre-SCT IA. One-year relapse-free survival was inferior in patients with pre-SCT IA (59.4% [48.3–68.9] vs. 70.4 [67.9–72.8]; multivariate HR 1.5 [1.1–2.1]; p = 0.02). Consequently, 1-year overall survival was lower in patients with pre-SCT IA: (68.8% [57.8–77.4] vs. 79.0% [76.7–81.1]; multivariate HR 1.7 [1.1–2.5]; p = 0.01). Interpretation: Pre-SCT IA remains to be significantly associated with impaired alloSCT outcome. On the other hand, more than two thirds of patients with pre-SCT IA were alive at one year after alloSCT. IA is not anymore an absolute contraindication for alloSCT because the majority of patients with IA who undergo alloSCT benefit from this procedure. Funding: There was no external funding source for this study.

Published
2024
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2. Improved outcomes over time and higher mortality in CMV seropositive allogeneic stem cell transplantation patients with COVID-19; An infectious disease working party study from the European Society for Blood and Marrow Transplantation registry

Author

Per Ljungman, Gloria Tridello, Jose Luis Piñana, Fabio Ciceri, Henrik Sengeloev, Alexander Kulagin, Stephan Mielke, Zeynep Arzu Yegin, Matthew Collin, Sigrun Einardottir, Sophie Ducastelle Lepretre, Johan Maertens, Antonio Campos, Elisabetta Metafuni, Herbert Pichler, Frantisek Folber, Carlos Solano, Emma Nicholson, Meltem Kurt Yüksel, Kristina Carlson, Beatriz Aguado, Caroline Besley, Jenny Byrne, Immaculada Heras, Fiona Dignan, Nicolaus Kröger, Christine Robin, Anjum Khan, Stig Lenhoff, Anna Grassi, Veronika Dobsinska, Nuno Miranda, Maria-Jose Jimenez, Ipek Yonal-Hindilerden, Keith Wilson, Dina Averbuch, Simone Cesaro, Alienor Xhaard, Nina Knelange, Jan Styczynski, Malgorzata Mikulska, and Rafael de la Camara

Subjects
COVID-19, allogeneic, stem cell transplantation, CMV, risk factors, Immunologic diseases. Allergy, RC581-607
Abstract

IntroductionCOVID-19 has been associated with high morbidity and mortality in allogeneic hematopoietic stem cell transplant (allo-HCT) recipients.MethodsThis study reports on 986 patients reported to the EBMT registry during the first 29 months of the pandemic.ResultsThe median age was 50.3 years (min – max; 1.0 – 80.7). The median time from most recent HCT to diagnosis of COVID-19 was 20 months (min – max; 0.0 – 383.9). The median time was 19.3 (0.0 - 287.6) months during 2020, 21.2 (0.1 - 324.5) months during 2021, and 19.7 (0.1 – 383.9) months during 2022 (p = NS). 145/986 (14.7%) patients died; 124 (12.6%) due to COVID-19 and 21 of other causes. Only 2/204 (1%) fully vaccinated patients died from COVID-19. There was a successive improvement in overall survival over time. In multivariate analysis, increasing age (p

Published
2023
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3. The urinary excretion of epigenetically modified DNA as a marker of pediatric ALL status and chemotherapy response

Author

Rafal Rozalski, Daniel Gackowski, Aleksandra Skalska-Bugala, Marta Starczak, Agnieszka Siomek-Gorecka, Ewelina Zarakowska, Martyna Modrzejewska, Tomasz Dziaman, Anna Szpila, Kinga Linowiecka, Jolanta Guz, Justyna Szpotan, Maciej Gawronski, Anna Labejszo, Lidia Gackowska, Marek Foksinski, Elwira Olinska, Aleksandra Wasilow, Andrzej Koltan, Jan Styczynski, and Ryszard Olinski

Subjects
Medicine, Science
Abstract

Abstract The active DNA demethylation process may be linked to aberrant methylation and may be involved in leukemogenesis. We investigated the role of epigenetic DNA modifications in childhood acute lymphoblastic leukemia (ALL) diagnostics and therapy monitoring. We analyzed the levels of 5-methyl-2′-deoxycytidine (5-mdC) oxidation products in the cellular DNA and urine of children with ALL (at diagnosis and during chemotherapy, n = 55) using two-dimensional ultra-performance liquid chromatography with tandem mass spectrometry (2D UPLC–MS/MS). Moreover, the expression of Ten Eleven Translocation enzymes (TETs) at the mRNA and protein levels was determined. Additionally, the ascorbate level in the blood plasma was analyzed. Before treatment, the ALL patients had profoundly higher levels of the analyzed modified DNA in their urine than the controls. After chemotherapy, we observed a statistically significant decrease in active demethylation products in urine, with a final level similar to the level characteristic of healthy children. The level of 5-hmdC in the DNA of the leukocytes in blood of the patient group was significantly lower than that of the control group. Our data suggest that urinary excretion of epigenetic DNA modification may be a marker of pediatric ALL status and a reliable marker of chemotherapy response.

Published
2021
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4. Short-term and mid-term effects of thoracoscopic repair of esophageal atresia: No anastomotic leaks or conversions to open technique

Author

Przemyslaw Galazka, Dominika Skinder, and Jan Styczynski

Subjects
esophageal atresia, thoracoscopic repair, newborn surgery, tracheoesophageal fistula, esophageal anastomosis, risk factors, Surgery, RD1-811
Abstract

The frequency rate of esophageal anastomosis leaks after thoracoscopic correction of esophageal atresia (EA) in the current literature is reported as 5.6%–24.7% and a conversion rate of 2%–53%. The objective of this retrospective study was to examine the characteristics of EA and analysis of the safety and efficacy of EA repair with the use of the thoracoscopic approach in a single academic center, as well as risk factors analysis in the context of short-term and mid-term follow-up status. A retrospective analysis of the management of all consecutive newborns affected by EA hospitalized in our department over a period between 2013 and 2022, including preoperative, perioperative, and postoperative management, together with the outcome, complications and long-term follow-up status was performed. A total of 38 patients with a median birth weight of 2,570 g (range; 1,020–3,880) were treated over the study period, including 30/38 (78.9%) with additional congenital anomalies. Overall, 30 patients underwent primary anastomosis of the esophagus and eight underwent a multistaged procedure, with or without an initial ligation of the tracheoesophageal fistula and delayed primary anastomosis. Overall survival for all patients was 0.894 ± 0.050, with a median follow-up of 4.5 years. We noted neither anastomotic leaks nor conversions to open technique in our cohort. Implementation of vancomycin prophylaxis was successful in preventing postoperative central venous access-related infectious complications. At the end of the follow-up, 85% of patients have a Lansky performance score ≥80. Risk factors analysis for length of hospitalization, overall survival, Lansky performance status, and neurological impairment were analyzed. In conclusion, we have found that the outcome of thoracoscopic repair of EA in terms of surgery-dependent morbidity (anastomosis leakage, conversion rate to open surgery), provides benefit to those previously reported in the literature, regardless of the prognostic criteria of the classification system.

Published
2022
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5. Improved outcome of patients with graft-versus-host disease after allogeneic hematopoietic cell transplantation for hematologic malignancies over time: an EBMT mega-file study

Author

Hildegard T. Greinix, Dirk-Jan Eikema, Linda Koster, Olaf Penack, Ibrahim Yakoub-Agha, Silvia Montoto, Christian Chabannon, Jan Styczynski, Arnon Nagler, Marie Robin, Stephen Robinson, Yves Chalandon, Malgorzata Mikulska, Stefan Schönland, Zinaida Peric, Annalisa Ruggeri, Francesco Lanza, Liesbeth C. de Wreede, Mohamad Mohty, Grzegorz W. Basak, and Nicolaus Kröger

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Acute graft-versus-host disease (aGvHD) remains a major threat to successful outcome following allogeneic hematopoietic cell transplantation though advances in prophylaxis and supportive care have been made. The aim of this study is to test whether the incidence and mortality of aGvHD have decreased over time. 102,557 patients with a median age of 47.6 years and with malignancies after first allogeneic sibling or unrelated donor (URD) transplant were studied in the following periods: 1990-1995, 1996-2000, 2001-2005, 2006-2010 and 2011-2015. Findings: 100-day incidences of aGvHD grades II-IV decreased from 40% to 38%, 32%, 29% and 28%, respectively, over calendar time (P

Published
2021
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6. Complications of Autologous Stem Cell Transplantation in Multiple Myeloma: Results from the CALM Study

Author

Anna Waszczuk-Gajda, Olaf Penack, Giulia Sbianchi, Linda Koster, Didier Blaise, Péter Reményi, Nigel Russell, Per Ljungman, Marek Trneny, Jiri Mayer, Simona Iacobelli, Guido Kobbe, Christof Scheid, Jane Apperley, Cyrille Touzeau, Stig Lenhoff, Esa Jantunen, Achilles Anagnostopoulos, Laura Paris, Paul Browne, Catherine Thieblemont, Nicolaas Schaap, Jorge Sierra, Ibrahim Yakoub-Agha, Laurent Garderet, Jan Styczynski, Helene Schoemans, Ivan Moiseev, Rafael F. Duarte, Zinaida Peric, Silvia Montoto, Anja van Biezen, Malgorzata Mikulska, Mahmoud Aljurf, Tapani Ruutu, Nicolaus Kröger, Curly Morris, Christian Koenecke, Stefan Schoenland, and Grzegorz W. Basak

Subjects
autologous stem cell transplantation in multiple myeloma, complications, multiple myeloma, Medicine
Abstract

Background: The main goal of this post hoc analysis of the Collaboration to Collect Autologous Transplant Outcomes in Lymphoma and Myeloma (CALM) study was to evaluate the rate of short- and long-term infectious and non-infectious complications occurring after ASCT in patients with multiple myeloma (MM). Methods: The analysis included all patients with MM from the CALM study who underwent ≥1 ASCT. The primary endpoint of the analysis was to determine the rate of infectious and non-infectious complications after ASCT and to compare them in three time periods: 0–100 days, 101 days–1 year, and >1 year after the first transplant. Results: The analysis included a total of 3552 patients followed up for a median of 56.7 months (range 0.4–108.1). Complication rates decreased with the time from ASCT with 24.85 cases per 100 patient-years from day 0 to 100 days after the transplant, and

Published
2022
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7. Management of adults and children undergoing chimeric antigen receptor T-cell therapy: best practice recommendations of the European Society for Blood and Marrow Transplantation (EBMT) and the Joint Accreditation Committee of ISCT and EBMT (JACIE)

Author

Ibrahim Yakoub-Agha, Christian Chabannon, Peter Bader, Grzegorz W. Basak, Halvard Bonig, Fabio Ciceri, Selim Corbacioglu, Rafael F. Duarte, Hermann Einsele, Michael Hudecek, Marie José Kersten, Ulrike Köhl, Jürgen Kuball, Stephan Mielke, Mohamad Mohty, John Murray, Arnon Nagler, Stephen Robinson, Riccardo Saccardi, Fermin Sanchez-Guijo, John A. Snowden, Micha Srour, Jan Styczynski, Alvaro Urbano-Ispizua, Patrick J. Hayden, and Nicolaus Kröger

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Chimeric antigen receptor (CAR) T cells are a novel class of anti-cancer therapy in which autologous or allogeneic T cells are engineered to express a CAR targeting a membrane antigen. In Europe, tisagenlecleucel (Kymriah™) is approved for the treatment of refractory/relapsed acute lymphoblastic leukemia in children and young adults as well as relapsed/refractory diffuse large B-cell lymphoma, while axicabtagene ciloleucel (Yescarta™) is approved for the treatment of relapsed/refractory high-grade B-cell lymphoma and primary mediastinal B-cell lymphoma. Both agents are genetically engineered autologous T cells targeting CD19. These practical recommendations, prepared under the auspices of the European Society of Blood and Marrow Transplantation, relate to patient care and supply chain management under the following headings: patient eligibility, screening laboratory tests and imaging and work-up prior to leukapheresis, how to perform leukapheresis, bridging therapy, lymphodepleting conditioning, product receipt and thawing, infusion of CAR T cells, short-term complications including cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome, antibiotic prophylaxis, medium-term complications including cytopenias and B-cell aplasia, nursing and psychological support for patients, long-term follow-up, post-authorization safety surveillance, and regulatory issues. These recommendations are not prescriptive and are intended as guidance in the use of this novel therapeutic class.

Published
2020
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8. Management of Epstein-Barr Virus infections and post-transplant lymphoproliferative disorders in patients after allogeneic hematopoietic stem cell transplantation: Sixth European Conference on Infections in Leukemia (ECIL-6) guidelines

Author

Jan Styczynski, Walter van der Velden, Christopher P. Fox, Dan Engelhard, Rafael de la Camara, Catherine Cordonnier, and Per Ljungman

Subjects
Diseases of the blood and blood-forming organs, RC633-647.5
Abstract

Epstein-Barr virus-related post-transplant lymphoproliferative disorders are recognized as a significant cause of morbidity and mortality in patients undergoing hematopoietic stem cell transplantation. To better define current understanding of post-transplant lymphoproliferative disorders in stem cell transplant patients, and to improve its diagnosis and management, a working group of the Sixth European Conference on Infections in Leukemia 2015 reviewed the literature, graded the available quality of evidence, and developed evidence-based recommendations for diagnosis, prevention, prophylaxis and therapy of post-transplant lymphoproliferative disorders exclusively in the stem cell transplant setting. The key elements in diagnosis include non-invasive and invasive methods. The former are based on quantitative viral load measurement and imaging with positron emission tomography; the latter with tissue biopsy for histopathology and detection of Epstein-Barr virus. The diagnosis of post-transplant lymphoproliferative disorder can be established on a proven or probable level. Therapeutic strategies include prophylaxis, preemptive therapy and targeted therapy. Rituximab, reduction of immunosuppression and Epstein-Barr virus-specific cytotoxic T-cell therapy are recommended as first-line therapy, whilst unselected donor lymphocyte infusions or chemotherapy are options as second-line therapy; other methods including antiviral drugs are discouraged.

Published
2016
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9. Endemic or regionally limited parasitic and fungal infections in haematopoietic stem-cell transplantation recipients: a Worldwide Network for Blood and Marrow Transplantation (WBMT) Review

Author

Ibrahim N Muhsen, Sebastian Galeano, Dietger Niederwieser, Mickey B C Koh, Per Ljungman, Clarisse M Machado, Mohamed A Kharfan-Dabaja, Rafael de la Camara, Yoshihisa Kodera, Jeff Szer, Walid Rasheed, Simone Cesaro, Shahrukh K Hashmi, Adriana Seber, Yoshiko Atsuta, Mostafa F Mohammed Saleh, Alok Srivastava, Jan Styczynski, Abdulrahman Alrajhi, Reem Almaghrabi, Muhammad Bilal Abid, Roy F Chemaly, Usama Gergis, Eolia Brissot, Riad El Fakih, Marcie Riches, Malgorzata Mikulska, Nina Worel, Daniel Weisdorf, Hildegard Greinix, Catherine Cordonnier, and Mahmoud Aljurf

Subjects
Hematology
Published
2023

10. Endemic or regionally limited bacterial and viral infections in haematopoietic stem-cell transplantation recipients: a Worldwide Network for Blood and Marrow Transplantation (WBMT) Review

Author

Ibrahim N Muhsen, Sebastian Galeano, Dietger Niederwieser, Mickey B C Koh, Per Ljungman, Clarisse M Machado, Mohamed A Kharfan-Dabaja, Rafael de la Camara, Yoshihisa Kodera, Jeff Szer, Walid Rasheed, Simone Cesaro, Shahrukh K Hashmi, Adriana Seber, Yoshiko Atsuta, Mostafa F Mohammed Saleh, Alok Srivastava, Jan Styczynski, Abdulrahman Alrajhi, Reem Almaghrabi, Muhammad Bilal Abid, Roy F Chemaly, Usama Gergis, Eolia Brissot, Riad El Fakih, Marcie Riches, Malgorzata Mikulska, Nina Worel, Daniel Weisdorf, Hildegard Greinix, Catherine Cordonnier, and Mahmoud Aljurf

Subjects
Hematology
Published
2023

12. Risk factors for a severe disease course in children with SARS-COV-2 infection following hematopoietic cell transplantation in the pre-Omicron period: a prospective multinational Infectious Disease Working Party from the European Society for Blood and Marrow Transplantation group (EBMT) and the Spanish Group of Hematopoietic Stem Cell Transplantation (GETH) study

Author

Dina Averbuch, Rafael de la Camara, Gloria Tridello, Nina Simone Knelange, Tatiana A. Bykova, Marianne Ifversen, Veronika Dobsinska, Mouhab Ayas, Amir Ali Hamidieh, Herbert Pichler, Antonio Perez-Martinez, Simone Cesaro, Mikael Sundin, Isabel Badell, Peter Bader, Jan-Erik Johansson, Oana Mirci-Danicar, Petr Sedlacek, Catherine Paillard, Brenda Gibson, Sarah Lawson, Nicolaus Kroeger, Selim Corbacioglu, Malgorzata Mikulska, Jose Luis Piñana, Jan Styczynski, and Per Ljungman

Subjects
Transplantation, Hematology
Published
2023

14. Mycobacterial infections in adults with haematological malignancies and haematopoietic stem cell transplants: guidelines from the 8th European Conference on Infections in Leukaemia

Author

Anne Bergeron, Malgorzata Mikulska, Julien De Greef, Louise Bondeelle, Tomas Franquet, Jean-Louis Herrmann, Christoph Lange, Isabel Spriet, Murat Akova, J Peter Donnelly, Johan Maertens, Georg Maschmeyer, Montserrat Rovira, Delia Goletti, Rafael de la Camara, Hildegard Greinix, Monica Slavin, Petr Hubacek, Catherine Cordonnier, Jukka Kanerva, Raoul Herbrecht, Fanny Lanternier, Christine Robin, Hermann Einsele, Thomas Lehrnbecher, Andreas Groll, Marie von Lilienfeld-Toal, Dorothea Pana, Emmanuel Roilides, Csaba Kassa, Diana Averbuch, Dan Engelhard, Simone Cesaro, Livio Pagano, Elio Castagnola, Francesca Compagno, Alessio Mesini, Peter J Donnelly, Jan Styczynski, Aida Botelho de Sousa, Mahmoud Aljurf, David Navarro, Carol Garcia-Vidal, Per Ljungman, Karlis Paukssen, Roland Ammann, Frédéric Lamoth, Hans Hirsch, Nicole Ritz, Mansour Ceesay, Adilia Warris, and Roy Chemaly

Subjects
Adult, Immunocompromised Host, Leukemia, Infectious Diseases, Hematologic Neoplasms, Hematopoietic Stem Cell Transplantation, Humans, Tuberculosis
Abstract

Mycobacterial infections, both tuberculosis and nontuberculous, are more common in patients with haematological malignancies and haematopoietic stem cell transplant recipients than in the general population-although these infections remain rare. Mycobacterial infections pose both diagnostic and therapeutic challenges. The management of mycobacterial infections is particularly complicated for patients in haematology because of the many drug-drug interactions between antimycobacterial drugs and haematological and immunosuppressive treatments. The management of mycobacterial infections must also consider the effect of delaying haematological management. We surveyed the management practices for latent tuberculosis infection (LTBI) in haematology centres in Europe. We then conducted a meticulous review of the literature on the epidemiology, diagnosis, and management of LTBI, tuberculosis, and nontuberculous mycobacterial infections among patients in haematology, and we formulated clinical guidelines according to standardised European Conference on Infections in Leukaemia (ECIL) methods. In this Review, we summarise the available literature and the recommendations of ECIL 8 for managing mycobacterial infections in patients with haematological malignancies.

Published
2022

16. Improved Outcome in Pediatric Acute Myeloid Leukemia: Progress With Hematopoietic Cell Transplantation

Author

Joanna, Stankiewicz and Jan, Styczynski

Subjects
Leukemia, Myeloid, Acute, Cancer Research, Treatment Outcome, Oncology, Recurrence, Hematopoietic Stem Cell Transplantation, Humans, General Medicine, Child
Abstract

Pediatric acute myeloid leukemia (AML) is a heterogenous disease; however, development of diagnostic methods has led to a better understanding of its nature and improvement in therapy outcomes. In this study, we evaluated treatment results in children with AML treated in a single oncology center in comparison with international results.Data from 77 children with AML treated in a single oncology center between 1994 and 2020 were analyzed. Patients had been treated according to five consecutive therapy protocols: AML NOPHO 88; ANLL 98; BFM AML 2004; BFM AML 2012, and BFM AML 2019. Five-year overall survival (OS), 5-year event-free survival (EFS) and 5-year relapse-free survival (RFS) were evaluated for each therapy protocol to determine prognostic factors and clarify differences between international and individual center results.During the observational period, 5-year OS increased from 55.6% to 85.7%, 5-year EFS from 45.7% to 87.5% and 5-year RFS from 51.4% to 85.7%. Hematopoietic cells transplantation (HCT) introduction in early 2000' improved treatment outcomes in the high-risk group, what has been mostly seen in the results of 5-year EFS. Treatment-related mortality was the most frequent cause of death in the analyzed group.Despite the significant improvement in therapy of pediatric AML, treatment outcomes remain unsatisfying. Introduction of HCT relevantly improved therapy results, especially in the high-risk group. International cooperation is crucial because of the small patient numbers in individual oncology centers.

Published
2022

17. Decrease of lethal infectious complications in the context of causes of death (COD) after hematopoietic cell transplantation: COD-2 and COD-1 study of the Infectious Diseases Working Party EBMT

Author

Jan Styczynski, Gloria Tridello, Linda Koster, Nina Knelange, Lotus Wendel, Anja van Biezen, Steffie van der Werf, Malgorzata Mikulska, Lidia Gil, Catherine Cordonnier, Per Ljungman, Diana Averbuch, Simone Cesaro, Helen Baldomero, Christian Chabannon, Selim Corbacioglu, Harry Dolstra, Bertram Glass, Raffaella Greco, Nicolaus Kröger, Régis Peffault de Latour, Mohamad Mohty, Benedicte Neven, Zinaida Peric, John A. Snowden, Anna Sureda, Ibrahim Yakoub-Agha, and Rafael de la Camara

Subjects
Transplantation, Hematology
Published
2023

18. Supplementary figure 2 from Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome

Author

Wojciech Mlynarski, Krystyna Chrzanowska, Hanna Gregorek, Bendik Lund, Jochen Buechner, Johann Greil, Alexandra Kreins, Ewa Wiesik-Szewczyk, Agnieszka Tomaszewska, Sara S. Kilic, Markus G. Seidel, Peter Svec, Mary Eapen, Dmitry Balashov, Michael H. Albert, Sujal Ghosh, Karl-Walter Sykora, Andrew R. Gennery, Eva Hlavackova, Zdenka Krenova, Larysa Kostyuchenko, Elena Deripapa, Natalia Miakova, Alina Fedorova, Jan Styczynski, Jolanta Gozdzik, Sylwia Koltan, Monika Lejman, Katarzyna Drabko, Anna Pieczonka, Marek Ussowicz, Krzysztof Kałwak, Barbara Pietrucha, Barbara Piątosa, Edyta Heropolitanska-Pliszka, Bozena Dembowska-Baginska, Anna Wakulinska, Wojciech Fendler, Agata Pastorczak, and Beata Wolska-Kusnierz

Abstract

Overall survival of patients with Nijmegen breakage syndrome depending on the time when they were treated due to malignancies: before and after 2000 year.

Published
2023

19. Supplementary figure 1 from Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome

Author

Wojciech Mlynarski, Krystyna Chrzanowska, Hanna Gregorek, Bendik Lund, Jochen Buechner, Johann Greil, Alexandra Kreins, Ewa Wiesik-Szewczyk, Agnieszka Tomaszewska, Sara S. Kilic, Markus G. Seidel, Peter Svec, Mary Eapen, Dmitry Balashov, Michael H. Albert, Sujal Ghosh, Karl-Walter Sykora, Andrew R. Gennery, Eva Hlavackova, Zdenka Krenova, Larysa Kostyuchenko, Elena Deripapa, Natalia Miakova, Alina Fedorova, Jan Styczynski, Jolanta Gozdzik, Sylwia Koltan, Monika Lejman, Katarzyna Drabko, Anna Pieczonka, Marek Ussowicz, Krzysztof Kałwak, Barbara Pietrucha, Barbara Piątosa, Edyta Heropolitanska-Pliszka, Bozena Dembowska-Baginska, Anna Wakulinska, Wojciech Fendler, Agata Pastorczak, and Beata Wolska-Kusnierz

Abstract

The dynamics of primary cancer incidence among patients with NBS described using the three-stage joint-point model.

Published
2023

20. Supplementary Figure 3 from Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome

Author

Wojciech Mlynarski, Krystyna Chrzanowska, Hanna Gregorek, Bendik Lund, Jochen Buechner, Johann Greil, Alexandra Kreins, Ewa Wiesik-Szewczyk, Agnieszka Tomaszewska, Sara S. Kilic, Markus G. Seidel, Peter Svec, Mary Eapen, Dmitry Balashov, Michael H. Albert, Sujal Ghosh, Karl-Walter Sykora, Andrew R. Gennery, Eva Hlavackova, Zdenka Krenova, Larysa Kostyuchenko, Elena Deripapa, Natalia Miakova, Alina Fedorova, Jan Styczynski, Jolanta Gozdzik, Sylwia Koltan, Monika Lejman, Katarzyna Drabko, Anna Pieczonka, Marek Ussowicz, Krzysztof Kałwak, Barbara Pietrucha, Barbara Piątosa, Edyta Heropolitanska-Pliszka, Bozena Dembowska-Baginska, Anna Wakulinska, Wojciech Fendler, Agata Pastorczak, and Beata Wolska-Kusnierz

Abstract

Overall survival of patients with Nijmegen breakage syndrome after cancer diagnosis. Patients who underwent HSCT were marked with an asterix.

Published
2023

21. Supplementary Data legend from Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome

Author

Wojciech Mlynarski, Krystyna Chrzanowska, Hanna Gregorek, Bendik Lund, Jochen Buechner, Johann Greil, Alexandra Kreins, Ewa Wiesik-Szewczyk, Agnieszka Tomaszewska, Sara S. Kilic, Markus G. Seidel, Peter Svec, Mary Eapen, Dmitry Balashov, Michael H. Albert, Sujal Ghosh, Karl-Walter Sykora, Andrew R. Gennery, Eva Hlavackova, Zdenka Krenova, Larysa Kostyuchenko, Elena Deripapa, Natalia Miakova, Alina Fedorova, Jan Styczynski, Jolanta Gozdzik, Sylwia Koltan, Monika Lejman, Katarzyna Drabko, Anna Pieczonka, Marek Ussowicz, Krzysztof Kałwak, Barbara Pietrucha, Barbara Piątosa, Edyta Heropolitanska-Pliszka, Bozena Dembowska-Baginska, Anna Wakulinska, Wojciech Fendler, Agata Pastorczak, and Beata Wolska-Kusnierz

Abstract

Supplementary Data legend

Published
2023

22. Supplementary Table 1 from Hematopoietic Stem Cell Transplantation Positively Affects the Natural History of Cancer in Nijmegen Breakage Syndrome

Author

Wojciech Mlynarski, Krystyna Chrzanowska, Hanna Gregorek, Bendik Lund, Jochen Buechner, Johann Greil, Alexandra Kreins, Ewa Wiesik-Szewczyk, Agnieszka Tomaszewska, Sara S. Kilic, Markus G. Seidel, Peter Svec, Mary Eapen, Dmitry Balashov, Michael H. Albert, Sujal Ghosh, Karl-Walter Sykora, Andrew R. Gennery, Eva Hlavackova, Zdenka Krenova, Larysa Kostyuchenko, Elena Deripapa, Natalia Miakova, Alina Fedorova, Jan Styczynski, Jolanta Gozdzik, Sylwia Koltan, Monika Lejman, Katarzyna Drabko, Anna Pieczonka, Marek Ussowicz, Krzysztof Kałwak, Barbara Pietrucha, Barbara Piątosa, Edyta Heropolitanska-Pliszka, Bozena Dembowska-Baginska, Anna Wakulinska, Wojciech Fendler, Agata Pastorczak, and Beata Wolska-Kusnierz

Abstract

Supplementary Table 1

Published
2023

23. Global effort to evacuate Ukrainian children with cancer and blood disorders who have been affected by war

Author

Asya Agulnik, Roman Kizyma, Marta Salek, Marcin W Wlodarski, Mikhail Pogorelyy, Aleksandra Oszer, Taisiya Yakimkova, Yuliya Nogovitsyna, Malgorzata Dutkiewicz, Jean-Hugues Dalle, Uta Dirksen, Angelika Eggert, Ana Fernández-Teijeiro, Jeanette Greiner, Kathelijne Kraal, Alexandra Mueller, Lucie Sramkova, Marco Zecca, Paul H Wise, Wojciech Mlynarski, Meghana Avula, Mykhaylo V Adyrov, Pablo Berlanga, Christopher Andrew Blackwood, Eric Bouffet, Piotr Stefan Czauderna, Linda A de Koning, Nuno Jorge dos Reis Farinha, Whitney Baer Foster, Dylan Elizabeth Graetz, Sumit Gupta, Wolfgang Holter, Rachael Emma Hough, Khrystyna Kliuchkivska, Alexandra Kolenova, Julia Kołodrubiec, Daniel C Moreira, Sheena Teresa Mukkada, Iryna Mykychak, Anna Raciborska, Zeena S Salman, Andriy Sopilnyak, Sergiy Tyupa, Anna Vinitsky, Natalia Margarete Wobst, Beth Anne Miller, Suheir Subhi Rasul, Carlos Rodriguez-Galindo, Inna Alanbousi, Sarah Weeks Alexander, Anna Apel, Wioletta Anna Bal, Walentyna Aniela Balwierz, Luisa Basset-Salom, Daniel Bastardo Blanco, Karolina Jadwiga Bauer, Ildar T Bayazitov, Nickhill Hitesh Bhakta, Ewa Iwona Bien, Katarzyna Anna Bieniek, Sally Jane Blair, Khrystyna Ihorivna Bodak, Irina Michael Bordeianu, Joao Maria Braganca, Mihaela Silvia Bucurenci, Elżbieta Beata Budny, Andrii Budzyn, Christopher Carl Bumgardner, Raina Nichole Burditt, Victoria Grace Burnside Clapp, Viacheslav Bykov, Adela Cañete, Monica Carnelli, Elena Cela, Zuzanna Paulina Cepowska, Radoslaw Chaber, Anna Cherner-Drieux, Mariya Chubata, Heidi M Clough, Jolanta Czernicka - Siwecka, Krzysztof Czyzewski, Olha Dashchakovska, Bozenna Malgorzata Dembowska-Baginska, Katarzyna Derwich, Rachel Dommett, Olha Dorosh, Katarzyna Anna Drabko, Monica Desiree Dragomir, Michael Dworzak, Sergii Dyma, Julian Darocus Earl, Martin William English, Dmitry A Evseev, Becky S Farren, Nataliia Fedyk, Severyn Ferneza, Leeanna Elizabeth Fox Irwin, Robert Maciej Gałązkowski, Galyna Ganieva, Vasylyna Garanzha, Marina S Gelman, Jan Krzysztof Godzinski, Anne Francoise Goeres, Rodica Golban, Michael J Griksaitis, Michal Andrzej Hampel, Sara Grace Hastings, Delphine Liliane Heenen, Marcela C Hill, Igor Holiuk, Lukasz Marek Hutnik, Ninela Irga-Jaworska, Oleksandr Istomin, Szymon Lech Janczar, Arman Kacharian, Krzysztof Kalwak, Grażyna Malgorzata Karolczyk, Nataliia Mikolaivna Karpenko, Halyna Katsubo, Bernarda Jadwiga Kaznowska, Alex Kentsis, Petra Ketteler, Anita Kienesberger, Roman Kiselev, Zoryana Kizyma, Hryhorii Klymniuk, Yuliia Kostiuk, Tomasz Kowalik, Olena Kozlova, Vladyslav Kozubenko, Tetyana Kramar, Maryna Krawczuk-Rybak, Irina Kulemzina, Paulina Kurkowska, Andriy S Kuzyk, Ruth Lydia Ladenstein, Pawel Jozef Laguna, Alvaro Lassaletta, Kai Lehmberg, Oksana Leontieva, Serhii Liashenko, Loizos G Loizou, Sonia Anna Lucchetta, Matthew William Lupo, Lesya Lysytsia, Oleksandr Lysytsia, Katarzyna Anna Machnik, Jeff A Mainland, Katarzyna Ewa Matczak, Michal Jacek Matysiak, Pierre Mayeur, Anastasia A Minervina, Volha Mishkova, Agnieszka Joanna Mizia-Malarz, Andres Morales La Madrid, Lucas Moreno, Vadim P Moskvin, Katarzyna Maria Muszyńska-Rosłan, Akoya Janae Nelson, Tomasz Ociepa, Stefano Oltolini, Nataliia Onipko, Andrew Pappas, Amit B Patel, Alina Patrahau, Jennifer L Pauley, Yehor Pavlenko, Andrij Pavlovych, Jarosław Peregud-Pogorzelski, Marta Perek-Polnik, Vanesa Perez, Antonio Perez-Martinez, Yana Pikman, Graziano Pitozzi, Rui Gentil Portugal, Victoria Vita Posternak, Arcangelo Prete, Kathy Pritchard-Jones, Alessandra Radaelli, Tegan Reeves, Dirk Reinhardt, Andrey V Reshetnyak, Andrew Jacob Rider, Carmelo Rizzari, Damiano Rizzi, Karen Gabriela Rodriguez Hermosillo, Olena Ronenko, Aneta Olga Rostowska, Liudmyla Rudko, Firas Mohamed Sakaan, Nadezhda Sakhar, Natallia N Savva, Davide Scaccaglia, Elizabeth Hawthorne Schaeffer, Carina Ursula Schneider, Nicole Scobie, Olena Semeniuk, Roksoliana Shevchyk, Ana I Shuler, Stanislav Shvets, Szymon Pawel Skoczen, William John Smeal, Igor Sokolowski, Anna A Sonkin, Alla Ivanivna Stepanjuk, Andrea Spota, Jaroslav Sterba, Jan Styczynski, Olha Svintsova, Andriy V Synyuta, Tomasz Szczepanski, Paweł Kukiz Szczucinski, Bartosz Miroslaw Szmyd, Maria Tasso Cereceda, Alina Teliuk, Iwona Tomanek, Phoebe Topping, Montserrat Torrent, Joanna Trelińska, Olha Troyanovska, Elena Trubnikova, Lyudmila G Tsurkan, Iryna Tsymbalyuk-Voloshyn, Tomasz Franciszek Urasinski, Agnieszka Urbanek-Dadela, Nataliia Vasilieva, Aksana Vasilyeva, Jaime Verdú-Amorós, Natalia Vilcu-Bajurean, Leo Vinitsky, Giovanni Volpe, Oksana Vorobel, Jacek Tadeusz Wachowiak, Marcin Slawomir Wasiak, Lance Allan Wiedower, Lena Isolde Wuenschel, Mariusz Stanislaw Wysocki, Marina Yurieva, Anastasiia Zagurska, Stanislav S Zakharenko, Aelita V Zakharenko, Khrystyna Zapotochna, Joanna Emilia Zawitkowska, Agulnik, A, Kizyma, R, Salek, M, Wlodarski, M, Pogorelyy, M, Oszer, A, Yakimkova, T, Nogovitsyna, Y, Dutkiewicz, M, Dalle, J, Dirksen, U, Eggert, A, Fernandez-Teijeiro, A, Greiner, J, Kraal, K, Mueller, A, Sramkova, L, Zecca, M, Wise, P, Mlynarski, W, Avula, M, Adyrov, M, Berlanga, P, Blackwood, C, Bouffet, E, Czauderna, P, de Koning, L, dos Reis Farinha, N, Foster, W, Graetz, D, Gupta, S, Holter, W, Hough, R, Kliuchkivska, K, Kolenova, A, Kolodrubiec, J, Moreira, D, Mukkada, S, Mykychak, I, Raciborska, A, Salman, Z, Sopilnyak, A, Tyupa, S, Vinitsky, A, Wobst, N, Miller, B, Rasul, S, Rodriguez-Galindo, C, Alanbousi, I, Alexander, S, Apel, A, Bal, W, Balwierz, W, Basset-Salom, L, Bastardo Blanco, D, Bauer, K, Bayazitov, I, Bhakta, N, Bien, E, Bieniek, K, Blair, S, Bodak, K, Bordeianu, I, Braganca, J, Bucurenci, M, Budny, E, Budzyn, A, Bumgardner, C, Burditt, R, Burnside Clapp, V, Bykov, V, Canete, A, Carnelli, M, Cela, E, Cepowska, Z, Chaber, R, Cherner-Drieux, A, Chubata, M, Clough, H, Czernicka - Siwecka, J, Czyzewski, K, Dashchakovska, O, Dembowska-Baginska, B, Derwich, K, Dommett, R, Dorosh, O, Drabko, K, Dragomir, M, Dworzak, M, Dyma, S, Earl, J, English, M, Evseev, D, Farren, B, Fedyk, N, Ferneza, S, Fox Irwin, L, Galazkowski, R, Ganieva, G, Garanzha, V, Gelman, M, Godzinski, J, Goeres, A, Golban, R, Griksaitis, M, Hampel, M, Hastings, S, Heenen, D, Hill, M, Holiuk, I, Hutnik, L, Irga-Jaworska, N, Istomin, O, Janczar, S, Kacharian, A, Kalwak, K, Karolczyk, G, Karpenko, N, Katsubo, H, Kaznowska, B, Kentsis, A, Ketteler, P, Kienesberger, A, Kiselev, R, Kizyma, Z, Klymniuk, H, Kostiuk, Y, Kowalik, T, Kozlova, O, Kozubenko, V, Kramar, T, Krawczuk-Rybak, M, Kulemzina, I, Kurkowska, P, Kuzyk, A, Ladenstein, R, Laguna, P, Lassaletta, A, Lehmberg, K, Leontieva, O, Liashenko, S, Loizou, L, Lucchetta, S, Lupo, M, Lysytsia, L, Lysytsia, O, Machnik, K, Mainland, J, Matczak, K, Matysiak, M, Mayeur, P, Minervina, A, Mishkova, V, Mizia-Malarz, A, Morales La Madrid, A, Moreno, L, Moskvin, V, Muszynska-Roslan, K, Nelson, A, Ociepa, T, Oltolini, S, Onipko, N, Pappas, A, Patel, A, Patrahau, A, Pauley, J, Pavlenko, Y, Pavlovych, A, Peregud-Pogorzelski, J, Perek-Polnik, M, Perez, V, Perez-Martinez, A, Pikman, Y, Pitozzi, G, Portugal, R, Posternak, V, Prete, A, Pritchard-Jones, K, Radaelli, A, Reeves, T, Reinhardt, D, Reshetnyak, A, Rider, A, Rizzari, C, Rizzi, D, Rodriguez Hermosillo, K, Ronenko, O, Rostowska, A, Rudko, L, Sakaan, F, Sakhar, N, Savva, N, Scaccaglia, D, Schaeffer, E, Schneider, C, Scobie, N, Semeniuk, O, Shevchyk, R, Shuler, A, Shvets, S, Skoczen, S, Smeal, W, Sokolowski, I, Sonkin, A, Stepanjuk, A, Spota, A, Sterba, J, Styczynski, J, Svintsova, O, Synyuta, A, Szczepanski, T, Szczucinski, P, Szmyd, B, Tasso Cereceda, M, Teliuk, A, Tomanek, I, Topping, P, Torrent, M, Trelinska, J, Troyanovska, O, Trubnikova, E, Tsurkan, L, Tsymbalyuk-Voloshyn, I, Urasinski, T, Urbanek-Dadela, A, Vasilieva, N, Vasilyeva, A, Verdu-Amoros, J, Vilcu-Bajurean, N, Vinitsky, L, Volpe, G, Vorobel, O, Wachowiak, J, Wasiak, M, Wiedower, L, Wuenschel, L, Wysocki, M, Yurieva, M, Zagurska, A, Zakharenko, S, Zakharenko, A, Zapotochna, K, and Zawitkowska, J

Subjects
Neoplasms, Medizin, Ethnicity, cancer, Humans, war, MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Hematology, Child, Hematologic Diseases, blood disorder
Published
2022

25. 1464. Real-World (RW) Utilization Pattern of Letermovir in Adult Cytomegalovirus Seropositive Allogeneic Hematopoietic Stem Cell Transplant (HCT) Recipients: A Multicenter Retrospective Cohort Study from the European Society for Blood and Marrow Transplantation (EBMT) Registry

Author

Jan Styczynski, Gloria Tridello, Nina Knelange, Lotus Wendel, Alienor Xhaard, Ilaria Cutini, Patrizia Chiusolo, Georg-Nikolaus Franke, Sabina Kraus, Amit D Raval, Sanjay Merchant, and Rafael de la Camara

Subjects
Infectious Diseases, Oncology
Abstract

Background Letermovir (LET) prophylaxis in CMV seropositive (R+) allogeneic (allo) HCT recipients was approved in 2017 based on a Phase 3 randomized clinical trial (RCT) demonstrating lower rates of CMV infection, disease, and all-cause mortality among LET treated participants. While RCTs demonstrate causal effects between treatments and outcomes, RW data answer practical aspects of utilization such as time to initiation of LET and duration of use. The objective of this study was to assess LET RW utilization pattern in adult CMV R+ recipients of allo-HCT in a large cohort of multiple centers in Europe participating in the EBMT registry. Methods A retrospective observational cohort design was used to examine the RW utilization patterns of LET among CMV R+ of an allo-HCT in France, Germany & Italy. Inclusion criteria were CMV R+ recipients >18 years who received LET for any reason between Jan 1 2018- Dec 31, 2020 and had data available for at least until 100 days post-HCT. Results In this interim analysis, 138 allo-HCT recipients (52% male) with median age 55 years (range 19-72) received LET as primary prophylaxis. Donor/recipient CMV serostatus was D+/R+ 64% D-/R+ 36%. Donor type was 65% unrelated, 20% mismatched relative, 15% sibling. Standard conditioning was used in 56% and reduced-intensity in 44%. Peripheral blood stem cells were the source in 89%. T-cell depletion was performed in 48%. All centers followed WHO standardization guidelines for CMV viral load assay by PCR. Viral load was determined at time of LET initiation in 86% patients. Oral (PO) only LET formulation was used in 98% of patients (median 2 days post HCT; LET was initiated prior to HCT in 3% of the recipients). Centers indicated that intravenous (IV) formulation was administered only if patients could not tolerate PO. The median duration of PO use was 98 days. LET dose was 240 mg (86%) mostly in patients receiving cyclosporine, 480 mg (9%) or sequentially 240/480 mg (5%). Conclusion Some differences were observed in this interim RW LET analysis compared to the phase 3 RCT. Notably, patients began LET at a median of D+2 post HCT (compared to D+9 in the trial), and 98% received only PO LET (as compared to 26.5% receiving IV LET in the trial). Further studies are needed to determine drivers of LET utilization in RW settings. Disclosures Jan Styczynski, MD, PhD, MSD: Honoraria Amit D. Raval, PhD, Merck and Co., Inc.: Employee of Merck|Merck and Co., Inc.: Stocks/Bonds Sanjay Merchant, PhD, Merck & Co., Inc.: Stocks/Bonds Rafael de la Camara, MD, MSD: Honoraria.

Published
2022

26. Management of Resistant Post-transplant Lymphoproliferative Disorder: CAR-T Is a New Option

Author

JAN STYCZYNSKI, JAGODA SADLOK, TOMASZ STYCZYNSKI, AGATA MARJANSKA, and MONIKA RICHERT-PRZYGONSKA

Subjects
Brentuximab Vedotin, Cancer Research, Herpesvirus 4, Human, Epstein-Barr Virus Infections, Receptors, Chimeric Antigen, Nivolumab, Oncology, Humans, General Medicine, Rituximab, Lymphoproliferative Disorders
Abstract

This is a review of the therapeutic options for resistant/refractory post-transplant lymphoproliferative disorder (PTLD) in relation to Chimeric antigen receptor-T cell (CAR-T) therapy. Out of a number of possible future strategies for the treatment of PTLD, the following methods were implemented in real-world practice: anti-PD1 therapy with checkpoint inhibitor nivolumab, new anti-CD20 ofatumumab, brentuximab vedotin, and zanubrutinib. However, for all these innovative methods, only individual cases of successful treatment of rituximab-resistant Epstein-Barr Virus (EBV)-PTLD patients have been reported so far. CAR-T is an innovative method of treatment, based on genetic modification of receptors of T autologous lymphocytes, creating the "living drug". This therapy can be potent against resistant PTLD, which is a lymphoproliferation of B-lymphocytes. The published real-world data of 17 patients treated with CAR-T for PTLD indicate a success rate of 76.5%. There is development of innovative methods of treatment of resistant/refractory PTLD, with high rate of resolution after CAR-T therapy.

Published
2022

27. Hepcidin in Children and Adults with Acute Leukemia or Undergoing Hematopoietic Cell Transplantation: A Systematic Review

Author

Jan Styczynski, Monika Łęcka, and Artur Slomka

Subjects
Cancer Research, Oncology
Abstract

Objectives: The association between hepcidin and acute leukemia (AL) or hematopoietic cell transplantation (HCT) in children and adults remains obscure. We aimed to assess this potential relationship through a systematic review of observational studies. Methods: An electronic search of three databases, including PubMed, Scopus, and Web of Science Core Collection, was performed up to 31 March 2022. Two independent reviewers assessed the search results according to predetermined inclusion and exclusion criteria, following PRISMA guidelines. Results: Of the 3607 titles identified, 13 studies published between 2008 and 2021 met the inclusion criteria. Most studies included a moderate number of participants and controls and used enzyme-linked immunosorbent assay (ELISA) to determine serum hepcidin levels. The principal findings: (1) serum hepcidin levels in patients with AL or undergoing HCT are increased compared to controls, regardless of the patient’s age and the phase of disease treatment; (2) AL therapy and HCT significantly influence serum hepcidin levels; (3) serum hepcidin may predict a worse outcome in patients with AL and post-HCT. Conclusions: This systematic review provides an overview of observational studies that deal with the association of hepcidin with AL and HCT. Although disturbances in iron metabolism are common in AL and HCT, and hepcidin seems to play a cardinal role in their modulation, more extensive research is needed.

Published
2022

28. New trends in the management of cytomegalovirus infection after allogeneic hematopoietic cell transplantation: a survey of the Infectious Diseases Working Pary of EBMT

Author

Simone Cesaro, Per Ljungman, Gloria Tridello, Malgorzata Mikulska, Lotus Wendel, Jan Styczynski, Dina Averbuch, and Rafael de la Camara

Subjects
Transplantation, Hematology
Abstract

The management of cytomegalovirus (CMV) infection was assessed with a survey performed in 2020 by the Infectious Diseases Working Party of European Society for Blood and Marrow Transplantation (EBMT). One-hundred-eighty of the 579 EBMT centres (31%) responded. CMV monitoring with quantitative PCR for CMV-DNAemia was used by 97% of centres while the duration of monitoring was variable according to the patient immune recovery and the ongoing immunosuppressive therapy. CMV prophylaxis for high-risk patients was used in 56% (101) of centres: letermovir in 62 centres (61%), aciclovir/valaciclovir in 19 centres (19%), ganciclovir/valganciclovir in 17 centres (17%), foscarnet in 3 (3%). The most used trigger for pre-emptive therapy was a threshold of > 103 copies/ml or > 103 IU/ml, Ganciclovir/valganciclovir confirmed the preferred first line treatment both for pre-emptive and CMV disease therapy. CMV-cytotoxic T-cells were used mainly in the setting of relapsing/refractory CMV disease. Forty-eight centres reported CMV refractory/resistant infection due to mutated CMV strain. This survey showed that letermovir prophylaxis is adopted by more than half of centres using a prophylaxis approach for CMV infection. How letermovir prophylaxis will modify other important pillars of daily CMV management, such as frequency of CMV-DNAemia monitoring and preemptive therapy, remain a matter of investigation.

Published
2022

29. Management of Herpesvirus Infections in Hematopoietic Cell Transplant Recipients

Author

Jan Styczynski

Subjects
Preventive strategy, Hematopoietic cell, business.industry, medicine.medical_treatment, Screening assay, Immunosuppression, Asymptomatic, Targeted therapy, Transplantation, Cell therapy, 03 medical and health sciences, 0302 clinical medicine, 030220 oncology & carcinogenesis, Immunology, medicine, medicine.symptom, business, 030215 immunology
Abstract

Following primary infection, herpesviruses establish latency in infected individuals in the host cells and may reactivate upon external stimuli and during periods of immunosuppression. The objective of this paper was to the present current strategies on preventive and therapeutic management of infections with herpesviruses in recipients of hematopoietic cell transplantation. Strategies of antiviral management include prophylaxis, pre-emptive treatment and targeted treatment. Empirical therapy is not used in antiviral strategies. Prophylaxis can be done at universal (preventive strategy) and specific level. Universal prophylaxis includes non-pharmacologic methods of prevention of infection or reactivation. Risk-adapted specific prophylaxis includes use of specific antivirals or cellular therapy or other specific methods in order to prevent specific infection, in high-risk groups. Pre-emptive therapy means use of therapeutic approaches in asymptomatic infection, detected by a screening assay. Targeted therapy is used in established specific viral end-organ infections. The following sections of the paper refer to prophylaxis and treatment strategies, respectively, against CMV, EBV, HSV, VZV, HHV-6, HHV-7, and HHV-8 after allogeneic hematopoietic cell transplantation.

Published
2021

30. Recommendations for the management of COVID-19 in patients with haematological malignancies or haematopoietic cell transplantation, from the 2021 European Conference on Infections in Leukaemia (ECIL 9)

Author

Simone, Cesaro, Per, Ljungman, Malgorzata, Mikulska, Hans H, Hirsch, Marie, von Lilienfeld-Toal, Catherine, Cordonnier, Sylvain, Meylan, Varun, Mehra, Jan, Styczynski, Francesco, Marchesi, Caroline, Besson, Fausto, Baldanti, Raul Cordoba, Masculano, Gernot, Beutel, Herman, Einsele, Elie, Azoulay, Johan, Maertens, and Rafael, de la Camara

Subjects
Settore MED/15 - MALATTIE DEL SANGUE, Cancer Research, Leukemia, Oncology, SARS-CoV-2, Hematologic Neoplasms, Hematopoietic Stem Cell Transplantation, COVID-19, Humans, Hematology
Abstract

Severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) is a novel virus that spread worldwide from 2019 causing the Coronavirus disease 19 (COVID-19) pandemic. SARS-CoV-2 infection is characterised by an initial viral phase followed in some patients by a severe inflammatory phase. Importantly, immunocompromised patients may have a prolonged viral phase, shedding infectious viral particles for months, and absent or dysfunctional inflammatory phase. Among haematological patients, COVID-19 has been associated with high mortality rate in acute leukaemia, high risk-myelodysplastic syndromes, and after haematopoietic cell transplant and chimeric-antigen-receptor-T therapies. The clinical symptoms and signs were similar to that reported for the overall population, but the severity and outcome were worse. The deferral of immunodepleting cellular therapy treatments is recommended for SARS-CoV-2 positive patient, while in the other at-risk cases, the haematological treatment decisions must be weighed between individual risks and benefits. The gold standard for the diagnosis is the detection of viral RNA by nucleic acid testing on nasopharyngeal-swabbed sample, which provides high sensitivity and specificity; while rapid antigen tests have a lower sensitivity, especially in asymptomatic patients. The prevention of SARS-CoV-2 infection is based on strict infection control measures recommended for aerosol-droplet-and-contact transmission. Vaccinations against SARS-CoV-2 has shown high efficacy in reducing community transmission, hospitalisation and deaths due to severe COVID-19 disease in the general population, but immunosuppressed/haematology patients may have lower sero-responsiveness to vaccinations. Moreover, the recent emergence of new variants may require vaccine modifications and strategies to improve efficacy in these vulnerable patients. Beyond supportive care, the specific treatment is directed at viral replication control (antivirals, anti-spike monoclonal antibodies) and, in patients who need it, to the control of inflammation (dexamethasone, anti-Il-6 agents, and others). However, the benefit of all these various prophylactic and therapeutic treatments in haematology patients deserves further studies.

Published
2022

31. Current attitude to deferral of cellular therapy or nontransplant chemotherapy due to SARS‐CoV‐2 asymptomatic infection: Survey of Infectious Diseases Working Party EBMT

Author

Jan Styczynski, Simone Cesaro, Marie Lilienfeld‐Toal, Francesco Marchesi, Lidia Gil, Malgorzata Mikulska, Nina Knelange, Lotus Wendel, Diana Averbuch, and Rafael Camara

Subjects
Transplantation, Infectious Diseases, SARS-CoV-2, COVID-19, Humans, Asymptomatic Infections, Communicable Diseases, Immunotherapy, Adoptive
Abstract

The objective of the study was to assess the current clinical practice and the attitude toward deferral of HCT/chemotherapy in patients with hematological diseases in cases of asymptomatic patients with a positive assay for SARS-CoV-2. In August 2021, we performed a survey among EBMT centers regarding their attitude toward deferral of HCT/chemotherapy in patients with a positive PCR result. Centers were willing to defer the planned cellular therapy for patients with asymptomatic SARS-CoV-2 infection without previous COVID-19 disease, and patients who became asymptomatic after a previous COVID19 disease but persistently shed the virus, respectively, in case of high-risk allo-HCT (90.2%/76.9%), low-risk allo-HCT for malignant diseases (88.2%/83.7%), allo-HCT for nonmalignant diseases (91.0%/91.0%), auto-HCT (88.0%/79.8%), and CAR-T therapy (83.1%/81.4%). The respective rates toward deferral of noncellular therapy patients was lower for both groups of patients, and varied with the primary diagnosis and anti-malignant treatment. There is a relatively high rate of willingness to defer treatment in asymptomatic patients being positive for SARS-CoV-2, planned for cellular therapy, regardless of previous history of vaccination or COVID-19. The same approach is presented for most of patients before noncellular therapy. Nevertheless, each patient should be considered individually weighting risks and benefits.

Published
2022

32. Discrepancies in the management of Clostridioides difficile infections in patients after allogeneic haematopoietic cell transplantation : the results of the Infectious Diseases Working Party EBMT survey

Author

Agnieszka Piekarska, Lidia Gil, Malgorzata Mikulska, Patrycja Mensah-Glanowska, Giulia Sbianchi, Lotus Wendel, Nina Knelange, Diana Averbuch, Rafael de la Camara, and Jan Styczynski

Subjects
Transplantation, Clostridioides difficile, Clostridium Infections, Hematopoietic Stem Cell Transplantation, Humans, Transplantation, Homologous, Hematology, Communicable Diseases
Published
2022

33. Impact of prophylaxis with rituximab on EBV-related complications after allogeneic hematopoietic cell transplantation in children

Author

Agata Marjańska, Monika Pogorzała, Magdalena Dziedzic, Krzysztof Czyżewski, Monika Richert-Przygońska, Robert Dębski, Tomasz Bogiel, and Jan Styczyński

Subjects
EBV infection, PTLD, children, hematopoietic cell transplantation, prophylaxis, transplant outcomes, Immunologic diseases. Allergy, RC581-607
Abstract

BackgroundChildren undergoing allo-HCT are at high risk of EBV-related complications. The objective of the study was to analyze the impact of prophylactic post-transplant rituximab on EBV infection and EBV-PTLD in children after allo-HCT, to determine the risk factors for the development of EBV infection and EBV-PTLD and to determine their outcomes. Additionally, the impact of EBV-driven complications on transplant outcomes was analyzed.MethodsSingle center retrospective analysis of EBV-related complications in pediatric population undergoing allo-HCT, based on strategy of prophylaxis with rituximab. Overall 276 consecutive children, including 122 on prophylaxis, were analyzed for EBV-driven complications and transplant outcomes.ResultsProphylaxis with rituximab resulted in significant reduction of EBV infection (from 35.1% to 20.5%; HR=2.7; p

Published
2024
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34. Prognostic impact of Epstein-Barr virus serostatus in patients with nonmalignant hematological disorders undergoing allogeneic hematopoietic cell transplantation: the study of Infectious Diseases Working Party of the European Society for Blood and Marrow Transplantation

Author

Jan Styczynski, Gloria Tridello, Lidia Gil, Per Ljungman, Malgorzata Mikulska, Steffie van der Werf, Nina Simone Knelange, Diana Averbuch, Gerard Socié, Hendrik Veelken, Jean-Hugues Dalle, Mahmoud Aljurf, Alphan Kupesiz, Yves Bertrand, Abdelghani Tbakhi, Boris Afanasyev, Bruno Lioure, Hélène Labussière-Wallet, Xavier Poiré, Johan Maertens, Eefke Petersen, Patrice Chevallier, Noel Milpied, John A. Snowden, Ibrahim Yakoub-Agha, Jan Cornelissen, Nicolaas Schaap, Carlo Dufour, Regis Peffault de Latour, Arjan Lankester, and Simone Cesaro

Subjects
Oncology, medicine.medical_specialty, Acute leukemia, business.industry, Incidence (epidemiology), Bone marrow failure, Hematology, Disease, medicine.disease, Lymphoma, Transplantation, 03 medical and health sciences, 0302 clinical medicine, Graft-versus-host disease, hemic and lymphatic diseases, 030220 oncology & carcinogenesis, Internal medicine, medicine, Serostatus, business, 030215 immunology
Abstract

BackgroundIn patients with acute leukemia, lymphoma and chronic malignancies, donor and/or recipient Epstein-Barr virus (EBV) seropositive status increases the risk of development of chronic graft-versus-host disease (cGVHD) after allo-hematopoietic cell transplantation (allo-HCT), while it has no influence on other transplant outcomes. No data are available on the impact of EBV serostatus on transplant outcomes in patients with nonmalignant hematological disorders.ObjectiveWe analyzed the influence of the recipient's (R) and donor's (D) EBV serostatus on transplant outcomes (overall survival (OS); relapse-free survival (RFS); relapse incidence (RI); nonrelapse mortality (NRM); acute graft-versus-host disease (aGVHD); cGVHD) in patients with nonmalignant hematological disorders undergoing allo-HCT.Patients and MethodsA total of 2,355 allo-HCTs performed between 1997 and 2016 for acquired bone marrow failure or hemoglobinopathies were included in this retrospective Registry megafile Infectious Diseases Working Party of the European Society of Blood and Marrow Transplantation (IDWP-EBMT) study.ResultsDemographics: The median age of recipient was 17.7 years (range: 0–77), and 50.8% were children. 79.0% of recipients and 75.4% of donors were EBV-seropositive. 67.8% had HCT from a matched family donor, 4.6% from a mismatched family donor, and 27.6% from an unrelated donor (UD). T-cell depletion was performed in vivo and ex vivo in 82.2% and 6.6% of patients, respectively. Conditioning regimen was myeloablative in 63.7% and reduced intensity conditioning (RIC) in 36.3% of patients. The median follow-up was 4.7 years. Transplant outcomes: EBV-seropositive recipients in comparison with EBV-seronegative recipients had lower OS (85.4% vs. 88.4%, p = 0.035) and higher NRM (10.0% vs. 6.4%, p = 0.018). No other significant differences were found for: RI, RFS, and aGVHD or cGVHD with respect to EBV pretransplant serostatus donor and/or recipient. Multivariate analysis: A trend toward higher risk of development of cGVHD (HR = 1.31; p = 0.081) and better survival (HR = 0.78; p = 0.087) in allo-HCT from EBV-seropositive donors was found. Allo-HCT in EBV-seropositive recipients had a trend toward lower risk of development of cGVHD (HR = 0.75; p = 0.065). When four subgroups (R−/D−, R−/D+, R+/D−, R+/D+ EBV serology) were analyzed, the EBV serostatus had no significant impact on OS, RFS, RI, NRM and development of aGVHD or cGVHD.ConclusionsAllo-HCT from EBV-seropositive versus EBV-seronegative donors are at 31% higher risk of cGVHD in patients with nonmalignant hematological disorders undergoing allo-HCT; however this difference is nonsignificant in multivariate analysis.

Published
2020

35. „Ми з Вами' (We Stand with You) – saving child war refugees from Ukraine by haematopoietic stem cell transplantation and chimeric antigen receptor T-cell therapy in Poland

Author

Tomasz Jarmoliński, Anna Fałkowska, Krzysztof Czyżewski, Agnieszka Sobkowiak-Sobierajska, Jolanta Goździk, Iwona Malinowska, Katarzyna Drabko, Jan Styczyński, Jacek Wachowiak, Paweł Łaguna, Marek Ussowicz, Oleksandr Istomin, Oleksandr Lysytsia, Wojciech Młynarski, and Krzysztof Kałwak

Subjects
poland, ukraine, children war refugees, haematopoietic stem cell transplantation, car-t cell therapy., Pediatrics, RJ1-570
Published
2024
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36. Incidence of bacterial and fungal infections in Polish pediatric patients with acute lymphoblastic leukemia during the pandemic

Author

Joanna Zawitkowska, Katarzyna Drabko, Monika Lejman, Adrian Kowalczyk, Krzysztof Czyżewski, Magdalena Dziedzic, Kamila Jaremek, Patrycja Zalas-Więcek, Anna Szmydki-Baran, Łukasz Hutnik, Wojciech Czogała, Walentyna Balwierz, Iwona Żak, Małgorzata Salamonowicz-Bodzioch, Bernarda Kazanowska, Grażyna Wróbel, Jowita Frączkiewicz, Krzysztof Kałwak, Renata Tomaszewska, Tomasz Szczepański, Olga Zając-Spychała, Jacek Wachowiak, Marcin Płonowski, Maryna Krawczuk-Rybak, Aleksandra Królak, Tomasz Ociepa, Tomasz Urasiński, Filip Pierlejewski, Wojciech Młynarski, Justyna Urbańska-Rakus, Katarzyna Machnik, Sonia Pająk, Wanda Badowska, Tomasz Brzeski, Katarzyna Mycko, Hanna Mańko-Glińska, Agnieszka Urbanek-Dądela, Grażyna Karolczyk, Agnieszka Mizia-Malarz, Weronika Stolpa, Katarzyna Skowron-Kandzia, Jakub Musiał, Radosław Chaber, Ninela Irga-Jaworska, Ewa Bień, and Jan Styczyński

Subjects
Medicine, Science
Abstract

Abstract The most common complications related to the treatment of childhood acute lymphoblastic leukemia (ALL) are infections. The aim of the study was to analyze the incidence and mortality rates among pediatric patients with ALL who were treated in 17 Polish pediatric hematology centers in 2020–2021 during the pandemic. Additionally, we compared these results with those of our previous study, which we conducted in the years 2012–2017. The retrospective analysis included 460 patients aged 1–18 years with newly diagnosed ALL. In our study, 361/460 (78.5%) children were reported to have microbiologically documented bacterial infections during chemotherapy. Ten patients (2.8%) died due to sepsis. Fungal infections were reported in 99 children (21.5%), of whom five (5.1%) died due to the infection. We especially observed an increase in bacterial infections during the pandemic period compared to the previous study. The directions of our actions should be to consider antibiotic prophylaxis, shorten the duration of hospitalization, and educate parents and medical staff about complications (mainly infections) during anticancer therapy. It is necessary to continue clinical studies evaluating infection prophylaxis to improve outcomes in childhood ALL patients.

Published
2023
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37. Ethyl Acetate Fraction from Eleutherococcus divaricatus Root Extract as a Promising Source of Compounds with Anti-Hyaluronidase, Anti-Tyrosinase, and Antioxidant Activity but Not Anti-Melanoma Activity

Author

Jakub Gębalski, Milena Małkowska, Sylwia Wnorowska, Dorota Gawenda-Kempczyńska, Maciej Strzemski, Magdalena Wójciak, Artur Słomka, Jan Styczyński, and Daniel Załuski

Subjects
Eleutherococcus divaricatus, human hyaluronidase, human tyrosinase, metabolites, Organic chemistry, QD241-441
Abstract

Eleutherococcus divaricatus (Siebold and Zucc.) S. Y. Hu. has been used in Traditional Chinese Medicine (TCM) due to its anticancer, immunostimulant, and anti-inflammatory activities. However, its mechanism of action and chemical composition are still insufficiently understood and require more advanced research, especially for cases in which anti-inflammatory properties are beneficial. The aim of this study was to evaluate the impact of E. divaricatus root extracts and fractions on proinflammatory serum hyaluronidase and tyrosinase in children diagnosed with acute lymphoblastic leukemia. Antioxidant and anti-melanoma activities were also examined and correlated with metabolomic data. For the first time, we discovered that the ethyl acetate fraction significantly inhibits hyaluronidase activity, with mean group values of 55.82% and 63.8% for aescin used as a control. However, interestingly, the fraction showed no activity against human tyrosinase, and in A375 melanoma cells treated with a doxorubicin fraction, doxorubicin activity decreased. This fraction exhibited the most potent antioxidant activity, which can be attributed to high contents of polyphenols, especially caffeic acid (24 mg/g). The findings suggest an important role of the ethyl acetate fraction in hyaluronidase inhibition, which may additionally indicate its anti-inflammatory property. The results suggest that this fraction can be used in inflammatory-related diseases, although with precautions in cases of patients undergoing chemotherapy.

Published
2024
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38. Acute Pancreatitis in Pediatric Acute Lymphoblastic Leukemia (AcuPA Study): A Nationwide Survey in Poland

Author

Anna Morawiak, Małgorzata Salamonowicz-Bodzioch, Aleksandra Królak, Krzysztof Kałwak, Joanna Owoc-Lempach, Jerzy Kowalczyk, Joanna Zawitkowska, Tomasz Szczepański, Ninela Irga-Jaworska, Elżbieta Adamkiewicz-Drożyńska, Katarzyna Albrecht, Anna Szmydki-Baran, Walentyna Balwierz, Małgorzata Czogała, Jacek Wachowiak, Katarzyna Derwich, Wojciech Młynarski, Beata Zalewska-Szewczyk, Maryna Krawczuk-Rybak, Małgorzata Sawicka-Żukowska, Jan Styczyński, Andrzej Kołtan, Krzysztof Safranow, Tomasz Urasiński, and Tomasz Ociepa

Subjects
acute lymphoblastic leukemia, children, acute pancreatitis, risk factors, intensive chemotherapy, asparaginase, Neoplasms. Tumors. Oncology. Including cancer and carcinogens, RC254-282
Abstract

Purpose: This study aimed to identify the risk factors for acute pancreatitis (AP) and its impact on outcomes in Polish children treated for ALL. Methods: The study group included 2303 children receiving intensive chemotherapy for ALL. The group was divided into patients with at least one episode of AP and those who did not develop AP after treatment for ALL. Results: The cumulative incidence of AP in the study group was 4.08%. Older age was an independent risk factor for the development of AP (OR = 1.05; 95%CI = 1.006–1.098; p = 0.03). The overall mortality associated with AP was 2.13%. The probabilities of disease-free survival (p-DFS) and event-free survival (p-EFS) in both subgroups were 0.84 vs. 0.86, log-rank p = 0.65 and 0.75 vs. 0.80, log-rank p = 0.12, respectively. A total of 22 out of 94 patients (23.4%) with AP were re-exposed to asparaginase (ASP) during the subsequent treatment phases. Only one patient re-exposed to ASP (4.5%) developed a second episode of AP. There were no significant differences in p-DFS and p-EFS between patients re-exposed and not re-exposed to asparaginase (0.78 vs. 0.86, log-rank p = 0.27 and 0.63 vs. 0.79, log-rank p = 0.09, respectively). Conclusions: The incidence of AP in children with ALL is low and related to patients’ age. The development of AP does not seem to influence p-DFS and p-EFS in children with ALL. Recurrence of AP after re-exposure to asparaginase in patients with ALL and a history of AP is low (4.5%). Re-exposure to asparaginase after the first episode of AP does not improve either p-DFS or p-EFS in children with ALL.

Published
2024
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39. Infections with Klebsiella pneumoniae in Children Undergoing Anticancer Therapy or Hematopoietic Cell Transplantation: A Multicenter Nationwide Study

Author

Alicja Sękowska, Krzysztof Czyżewski, Kamila Jaremek, Patrycja Zalas-Więcek, Olga Zając-Spychała, Jacek Wachowiak, Anna Szmydki-Baran, Łukasz Hutnik, Agnieszka Gietka, Olga Gryniewicz-Kwiatkowska, Bożenna Dembowska-Bagińska, Katarzyna Semczuk, Katarzyna Dzierżanowska-Fangrat, Wojciech Czogała, Walentyna Balwierz, Iwona Żak, Renata Tomaszewska, Tomasz Szczepański, Ewa Bień, Ninela Irga-Jaworska, Katarzyna Machnik, Justyna Urbańska-Rakus, Sonia Pająk, Marcin Płonowski, Maryna Krawczuk-Rybak, Aleksandra Królak, Tomasz Ociepa, Tomasz Urasiński, Paweł Wawryków, Jarosław Peregud-Pogorzelski, Tomasz Brzeski, Katarzyna Mycko, Hanna Mańko-Glińska, Wanda Badowska, Agnieszka Urbanek-Dądela, Grażyna Karolczyk, Weronika Stolpa, Katarzyna Skowron-Kandzia, Agnieszka Mizia-Malarz, Filip Pierlejewski, Wojciech Młynarski, Jakub Musiał, Radosław Chaber, Joanna Zawitkowska, Agnieszka Zaucha-Prażmo, Katarzyna Drabko, Jolanta Goździk, Jowita Frączkiewicz, Małgorzata Salamonowicz-Bodzioch, Krzysztof Kałwak, and Jan Styczyński

Subjects
acute leukemia, children, hematopoietic cell transplantation, infection, Klebsiella pneumoniae, Medicine
Abstract

Background: Klebsiella pneumoniae is a nosocomial pathogen that causes severe infections in immunocompromised patients. The aim of the study was to conduct a microbiological and clinical analysis of K. pneumoniae infections in children with malignancies or undergoing hematopoietic cell transplantation in Poland. Methods: We conducted a retrospective, multicenter study including children and adolescents under 19 years old treated between 2012 and 2021. We analyzed patients’ characteristics, microbiological data, and the outcomes of antibiotic therapy. Results: A total of 9121 newly diagnosed children were treated for malignancy and 1697 pediatric patients underwent hematopoietic cell transplantation. K. pneumoniae infections were diagnosed in 527 patients. Their overall incidence was 4.86% in pediatric hematology and oncology patients and 4.95% in patients who underwent hematopoietic cell transplantation. The incidence of infection was higher in patients with acute leukemia than with solid tumors (7.8% vs. 4.1%; OR = 2.0; 95% CI = 1.6–2.4; p < 0.0001). The most frequent source of infection was in the urinary tract at 55.2%. More than 57% of K. pneumoniae strains were extended-spectrum β-lactamase-positive and almost 34% were multidrug-resistant. Infections with K. pneumoniae contributed to death in 3.22% of patients. Conclusions: K. pneumoniae is one of the most critical pathogens in children suffering from malignancies or undergoing hematopoietic cell transplantation. The incidence of multidrug-resistant K. pneumoniae strains is increasing and contributing to poor clinical outcome.

Published
2024
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40. Correction: Long-term outcome after allogeneic hematopoietic stem cell transplantation for Shwachman–Diamond syndrome: a retrospective analysis and a review of the literature by the Severe Aplastic Anemia Working Party of the European Society for Blood and Marrow Transplantation (SAAWP-EBMT)

Author

Simone Cesaro, Marta Pillon, Martin Sauer, Frans Smiers, Maura Faraci, Cristina Diaz de Heredia, Robert Wynn, Johann Greil, Franco Locatelli, Paul Veys, Anne Uyttebroeck, Per Ljungman, Patrice Chevalier, Marc Ansari, Isabel Badell, Tayfun Güngör, Rahuman Salim, Johanna Tischer, Cristina Tecchio, Nigel Russell, Alicja Chybicka, Jan Styczynski, Gergely Krivan, Owen Smith, Jerry Stein, Boris Afanasyev, Cécile Pochon, Maria Cristina Menconi, Paul Bosman, Margherita Mauro, Gloria Tridello, Regis Peffault de Latour, Carlo Dufour, and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)

Subjects
Transplantation, [SDV.MHEP.PED]Life Sciences [q-bio]/Human health and pathology/Pediatrics, ALLOGENEIC HEMATOPOIETIC STEM CELL TRANSPLANTATION, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Hematology, SAA, 3. Good health, 03 medical and health sciences, SWACHMAN DIAMOND SYNFROME, 0302 clinical medicine, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, 030220 oncology & carcinogenesis, ComputingMilieux_MISCELLANEOUS, 030215 immunology
Abstract

International audience

Published
2020

42. Correction: Benchmarking of survival outcomes following haematopoietic stem cell transplantation: A review of existing processes and the introduction of an international system from the European Society for Blood and Marrow Transplantation (EBMT) and the Joint Accreditation Committee of ISCT and EBMT (JACIE)

Author

John A. Snowden, Riccardo Saccardi, Kim Orchard, Per Ljungman, Rafael F. Duarte, Myriam Labopin, Eoin McGrath, Nigel Brook, Carmen Ruiz de Elvira, Debra Gordon, Hélène A. Poirel, Francis Ayuk, Yves Beguin, Francesca Bonifazi, Alois Gratwohl, Noel Milpied, John Moore, Jakob Passweg, J. Douglas Rizzo, Stephen R. Spellman, Jorge Sierra, Carlos Solano, Fermin Sanchez-Guijo, Nina Worel, Andreu Gusi, Gillian Adams, Theodor Balan, Helen Baldomero, Gilles Macq, Evelyne Marry, Florence Mesnil, Elena Oldani, Rachel Pearce, Julia Perry, Nicole Raus, Urs Schanz, Steven Tran, Leonie Wilcox, Grzegorz W. Basak, Christian Chabannon, Selim Corbacioglu, Harry Dolstra, Jürgen Kuball, Mohamad Mohty, Arjan Lankester, Sylvia Montoto, Arnon Nagler, Jan Styczynski, Ibrahim Yakoub-Agha, Regis Peffault de Latour, Nicolaus Kroeger, Ronald Brand, Liesbeth C. de Wreede, Erik van Zwet, and Hein Putter

Subjects
Haematological cancer, Transplantation, Haematopoietic stem cells, Australia, Hematopoietic Stem Cell Transplantation, Correction, Hematology, United Kingdom, Accreditation, Europe, Benchmarking, Belgium, Italy, Bone Marrow, Spain, Germany, Humans, France, Switzerland
Abstract

In many healthcare settings, benchmarking for complex procedures has become a mandatory requirement by competent authorities, regulators, payers and patients to assure clinical performance, cost-effectiveness and safe care of patients. In several countries inside and outside Europe, benchmarking systems have been established for haematopoietic stem cell transplantation (HSCT), but access is not universal. As benchmarking is now integrated into the FACT-JACIE standards, the EBMT and JACIE established a Clinical Outcomes Group (COG) to develop and introduce a universal system accessible across EBMT members. Established systems from seven European countries (United Kingdom, Italy, Belgium, France, Germany, Spain, Switzerland), USA and Australia were appraised, revealing similarities in process, but wide variations in selection criteria and statistical methods. In tandem, the COG developed the first phase of a bespoke risk-adapted international benchmarking model for one-year survival following allogeneic and autologous HSCT based on current capabilities within the EBMT registry core dataset. Data completeness, which has a critical impact on validity of centre comparisons, is also assessed. Ongoing development will include further scientific validation of the model, incorporation of further variables (when appropriate) alongside implementation of systems for clinically meaningful interpretation and governance aiming to maximise acceptance to centres, clinicians, payers and patients across EBMT.

Published
2019

43. Infectious complications in children with malignant bone tumors: a multicenter nationwide study

Author

Krzysztof, Czyzewski, Przemyslaw, Galazka, Patrycja, Zalas-Wiecek, Olga, Gryniewicz-Kwiatkowska, Agnieszka, Gietka, Katarzyna, Semczuk, Liliana, Chelmecka-Wiktorczyk, Iwona, Zak, Malgorzata, Salamonowicz, Jowita, Fraczkiewicz, Olga, Zajac-Spychala, Ewa, Bien, Marcin, Plonowski, Pawel, Wawrykow, Filip, Pierlejewski, Zuzanna, Gamrot, Zofia, Malas, Weronika, Stolpa, Jakub, Musial, and Jan, Styczynski

Subjects
infectious complications, risk factors analysis, children, osteosarcoma, Ewing sarcoma, Original Research, malignant bone tumor
Abstract

Objectives: The analysis of epidemiology, risk factors and outcome of infections in children with malignant bone tumors (MBT) undergoing chemotherapy. Methods: In this retrospective nationwide multicenter cross-sectional study, a total number of 126 children with MBT including 70 with Ewing sarcoma (ES) and 56 with osteosarcoma (OSA) were screened for infections over a period of 72 consecutive months. Results: The risk of infection was 7.15-fold higher in patients with ES as compared to the OSA group, especially concerning bacterial infections (4.1-fold increase risk). Bacterial infections occurred in 74.3% patients with ES and in 41.1% with OSA. The median time from diagnosis to first infection was 4.9 months. 33.0% of bacterial episodes were diagnosed as bloodstream (BSI), 31.1% as gastrointestinal tract, 30.1% as urinary tract infection. Infection-related mortality (IRM) from bacterial infection was 6% and 15% in ES and OSA patients, respectively. Cumulative incidence was 7.1% for invasive fungal disease and 6.3% for viral infections. The only significant risk factor for IRM was time to infection ≥5 months since the beginning of chemotherapy. All patients who have died from infection had BSI and were in neutropenia. Conclusions: Infections in the children with MBT in our study occurred with high frequency, especially in patients with ES. The most frequent were bacterial infections, while fungal and viral infections were episodic. Among the bacterial infections, bloodstream, urinary tract and gastrointestinal tract infections occurred with similar frequency. All deceased patients died due to BSI. Bacterial infection occurring ≥5 months since the beginning of chemotherapy was a risk factor for death.

Published
2018

44. Managing Undernutrition in Pediatric Oncology: A Consensus Statement Developed Using the Delphi Method by the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology

Author

Agnieszka Budka-Chrzęszczyk, Agnieszka Szlagatys-Sidorkiewicz, Ewa Bień, Ninela Irga-Jaworska, Anna Borkowska, Małgorzata Anna Krawczyk, Katarzyna Popińska, Hanna Romanowska, Ewa Toporowska-Kowalska, Magdalena Świder, Jan Styczyński, Tomasz Szczepański, and Janusz Książyk

Subjects
childhood cancer, nutrition, nutritional support, Nutrition. Foods and food supply, TX341-641
Abstract

“Managing Undernutrition in Pediatric Oncology” is a collaborative consensus statement of the Polish Society for Clinical Nutrition of Children and the Polish Society of Pediatric Oncology and Hematology. The early identification and accurate management of malnutrition in children receiving anticancer treatment are crucial components to integrate into comprehensive medical care. Given the scarcity of high-quality literature on this topic, a consensus statement process was chosen over other approaches, such as guidelines, to provide comprehensive recommendations. Nevertheless, an extensive literature review using the PubMed database was conducted. The following terms, namely pediatric, childhood, cancer, pediatric oncology, malnutrition, undernutrition, refeeding syndrome, nutritional support, and nutrition, were used. The consensus was reached through the Delphi method. Comprehensive recommendations aim to identify malnutrition early in children with cancer and optimize nutritional interventions in this group. The statement underscores the importance of baseline and ongoing assessments of nutritional status and the identification of the risk factors for malnutrition development, and it presents tools that can be used to achieve these goals. This consensus statement establishes a standardized approach to nutritional support, aiming to optimize outcomes in pediatric cancer patients.

Published
2024
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45. Results of allogeneic stem cell transplantation in patients affected by Shwachman-Diamond syndrome. A retrospective study of SAA-EBMT

Author

Cesaro, Simone, Gloria, Tridello, Christian, Kratz, Marta, Pillon, Halkes, S, Edoardo, Lanino, Regis Peffault De Latour, C Diaz De Heredia, Robert, Wynn, Johann, Greil, Franco, Locatelli, Paul, Veys, Anne, Uyttebroeck, Per, Ljungman, Patrice, Chevalier, Marc, Ansari, Isabel, Badell, Tayfun, Gungor, Salim, Rahuman, Johanna, Tischer, Tecchio, Cristina, Nigel, Russel, Alicja, Chybicka, Kallay, K, Owen, Smith, Afanasyev, B, Stein, J, Jan, Styczynski, Paul, Bosman, and Carlo, Dufour.

Subjects
Shwachman Diamond Disease, stem cell transplantation, survival, stem cell transplantation, survival, Shwachman Diamond Disease
Published
2018

46. Intercontinental study of Infectious Diseases Working Party of EBMT

Author

Diana, Averbuch, Gloria, Tridello, Jennifer, Hoek, Malgorzata, Mikulska4, Thomas, Pabst, Lucrecia Yaňez San Segundo, Hamdi, Akan, Tülay, Özçelik, Irene, Donnini, Galina, Klyasova, Aida Botelho de Sousa, Tsila, Zuckerman, Depei, Wu, Tecchio, Cristina, Rafael de la Camara, Sahika Zeynep Aki, Per, Ljungman, Zafer, Gülbas, Emmanuelle, Nicolas, Elisabetta, Calore, Katia, Perruccio, Ron, Ram, Claudio, Annaloro, Rodrigo, Martino, Batia, Avni, Peter, Shaw, Lidia, Gil, Simona, Iacobelli, Jan, Styczynski, Dan, Engelhard, and Cesaro, Simone

Subjects
infectious complications, stem cell transplantation, mortality, infectious complications, stem cell transplantation, mortality
Published
2018

47. Fluoroquinolone prophylaxis in haematological cancer patients with neutropenia: ECIL critical appraisal of previous guidelines

Author

Malgorzata Mikulska, Diana Averbuch, Frederic Tissot, Catherine Cordonnier, Murat Akova, Thierry Calandra, Marcello Ceppi, Paolo Bruzzi, Claudio Viscoli, Mahmoud Aljurf, Dina Averbuch, Rosemary Barnes, Ola Blennow, Pierre-Yves Bochud, Emilio Bouza, Stephane Bretagne, Roger Brüggemann, Jordi Carratala, Simone Cesaro, Oliver Cornely, Tina Dalianis, Rafael De La Camara, Peter Donnelly, Lubos Drgona, Rafael Duarte, Hermann Einsele, Dan Engelhard, Christopher Fox, Corrado Girmenia, Andreas Groll, Dag Heldal, Jannick Helweg Larsen, Raoul Herbrecht, Hans Hirsch, Elisabeth Johnson, Galina Klyasova, Minna Koskuenvo, Katrien Lagrou, Russel E. Lewis, Per Ljungman, Johan Maertens, Georg Maschmeyer, Marcio Nucci, Christophe Padoin, Livio Pagano, Antonio Pagliuca, Zdenek Racil, Patricia Ribaud, Christine Rinaldo, Valérie Rizzi Puechal, Emmanuel Roilides, Christine Robin, Montserrat Rovira, Markus Rupp, Sonia Sanchez, Peter Schellongowski, Peter Sedlacek, Janos Sinko, Monica Slavin, Isabella Sousa Ferreira, Jan Styczynski, Katherine Ward, Anne-Therese Witschi, Mikulska, Malgorzata, Averbuch, Diana, Tissot, Frederic, Cordonnier, Catherine, Akova, Murat, Calandra, Thierry, Ceppi, Marcello, Bruzzi, Paolo, Viscoli, Claudio, Aljurf, Mahmoud, Averbuch, Dina, Barnes, Rosemary, Blennow, Ola, Bochud, Pierre-Yve, Bouza, Emilio, Bretagne, Stephane, Brüggemann, Roger, Carratala, Jordi, Cesaro, Simone, Cornely, Oliver, Dalianis, Tina, De La Camara, Rafael, Donnelly, Peter, Drgona, Lubo, Duarte, Rafael, Einsele, Hermann, Engelhard, Dan, Fox, Christopher, Girmenia, Corrado, Groll, Andrea, Heldal, Dag, Larsen, Jannick Helweg, Herbrecht, Raoul, Hirsch, Han, Johnson, Elisabeth, Klyasova, Galina, Koskuenvo, Minna, Lagrou, Katrien, Lewis, Russel E., Ljungman, Per, Maertens, Johan, Maschmeyer, Georg, Nucci, Marcio, Padoin, Christophe, Pagano, Livio, Pagliuca, Antonio, Racil, Zdenek, Ribaud, Patricia, Rinaldo, Christine, Puechal, Valérie Rizzi, Roilides, Emmanuel, Robin, Christine, Rovira, Montserrat, Rupp, Marku, Sanchez, Sonia, Schellongowski, Peter, Sedlacek, Peter, Sinko, Jano, Slavin, Monica, Ferreira, Isabella Sousa, Styczynski, Jan, Ward, Katherine, and Witschi, Anne-Therese

Subjects
Neutropenic, Microbiology (medical), Ciprofloxacin, Febrile neutropenia, Infection, Levofloxacin, Multidrug resistance (MDR), Prevention, Quinolone, medicine.medical_specialty, Neutropenia, Guidelines as Topic, Infections, law.invention, 03 medical and health sciences, 0302 clinical medicine, Antibiotic resistance, Randomized controlled trial, law, Internal medicine, medicine, Humans, Ciprofloxacin, Febrile neutropenia, Infection, Levofloxacin, Multidrug resistance (MDR), Neutropenic, Prevention, Quinolone, Microbiology (medical), Infectious Diseases, 030212 general & internal medicine, Intensive care medicine, Infection Control, business.industry, Antibiotic Prophylaxis, medicine.disease, Anti-Bacterial Agents, Settore MED/15 - MALATTIE DEL SANGUE, Resistant bacteria, Critical appraisal, lnfectious Diseases and Global Health Radboud Institute for Health Sciences [Radboudumc 4], Infectious Diseases, Hematologic Neoplasms, 030220 oncology & carcinogenesis, Haematological cancer, Observational study, business, Inflammatory diseases Radboud Institute for Molecular Life Sciences [Radboudumc 5], Fluoroquinolones
Abstract

Contains fulltext : 190513.pdf (Publisher’s version ) (Closed access) OBJECTIVES: Fluoroquinolone (FQ) prophylaxis was recommended in 2005 by European Conference on Infections in Leukemia (ECIL) for patients with prolonged neutropenia. In consideration of a worldwide increase in antibiotic resistance, the issue of FQ prophylaxis during neutropenia was re-evaluated. METHODS: Literature review of randomised controlled trials (RCT) and observational studies published in years 2006-2014 was performed. Their results were analysed in meta-analysis. Meta-regression model was applied to evaluate whether the rates of FQ resistance in community and hospital settings influenced the efficacy of FQ prophylaxis. The impact of FQ prophylaxis on colonisation and infection with resistant bacteria was reviewed. RESULTS: Two RCTs and 12 observational studies were identified. FQ prophylaxis did not have effect on mortality (pooled OR 1.01, 95%CI 0.73-1.41), but was associated with lower rate of bloodstream infections (BSI) (pooled OR 0.57, 95%CI 0.43-0.74) and episodes of fever during neutropenia (pooled OR 0.32, 95%CI 0.20-0.50). No effect of the background rate of FQ resistance on the efficacy of FQ prophylaxis was observed. In few studies, FQ prophylaxis resulted in an increased colonisation or infection with FQ- or multi-drug resistant strains. CONCLUSIONS: The possible benefits of FQ prophylaxis on BSI rate, but not on overall mortality, should be weighed against its impact in terms of toxicity and changes in local ecology in single centres.

Published
2018

48. Lethal infectious complications after hematopoietic stem cell transplantation: progress and challenges in Infectious Diseases Working Party study

Author

Jan, Styczynski, Gloria, Tridello, Linda, Koster, Simona, Iacobelli, Anja van Biezen, Steffie van der Werf, Malgorzata, Mikulska, Lidia, Gil, Catherine, Cordonnier, Per, Ljungman, Diana, Averbuch, Cesaro, Simone, Rafael de la Camara, Helen, Baldomero, Peter, Bader, Grzegorz, Basak, Chiara, Bonini, Rafael, Duarte, Carlo, Dufour, Jurgen, Kuball, Arjan, Lankester, Silvia, Montoto, John, Snowden, Arnon, Nagler, Nicolaus, Kröger, Mohamad, Mohty, and Alois, Gratwoh.

Subjects
lethality, infection, lethality, stem cell transplantation, stem cell transplantation, infection
Published
2018

50. Pre-Exposure Prophylaxis and Treatment with Tixagevimab/Cilgavimab for COVID-19 among Immunocompromised Pediatric Patients

Author

Jowita Frączkiewicz, Katarzyna Pawińska-Wąsikowska, Katarzyna Szymbor, Walentyna Balwierz, Szymon Skoczeń, Krzysztof Czyżewski, Sylwia Kołtan, Jan Styczyński, Anna Małecka, Ninela Irga-Jaworska, Joanna Trelińska, Wojciech Młynarski, Olga Zając-Spychała, Agnieszka Sobkowiak-Sobierajska, Katarzyna Derwich, Wioletta Bal, Radosław Chaber, Agnieszka Książek, Tomasz Szczepański, Joanna Zawitkowska, Katarzyna Drabko, Agnieszka Chodała-Grzywacz, Grażyna Karolczyk, Christopher Kobierzycki, and Krzysztof Kałwak

Subjects
COVID-19, immunocompromised patients, monoclonal antibodies, tixagevimab, cilgavimab, evusheld, Medicine
Abstract

Background: Patients treated with hemato-oncological malignancies (HO) or undergoing cellular therapies such as hematopoietic stem cell transplantation (HSCT) or chimeric antigen receptor T cells (CAR-T) were significantly affected by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Despite the success of SARS-CoV-2 vaccination, immunocompromised patients remain at increased risk for severe coronavirus disease (COVID-19), rendering this group of population a high priority for additional prevention and treatment options. Tixagevimab and Cilgavimab (TIXA/CILGA, AZD7442, Evusheld®) is a combination of two fully human, long-acting monoclonal antibodies. TIXA/CILGA have been approved as pre-exposure prophylaxis and treatment in patients at risk of severe disease with impaired vaccine response. Our objective was to describe the efficacy and safety among immunocompromised pediatric patients. Methods: This was an observational multicenter cohort study of immunocompromised pediatric patients receiving TIXA/CILGA conducted at nine Polish centers of Pediatric Oncology, Hematology and Bone Marrow Transplantation. We analyzed patients in two groups; those treated with HO and those undergoing cellular therapies: HSCT or CAR-T cells. In addition, two other cohorts were identified: patients given TIXA/CILGA as pre-exposure prophylactic and therapeutic intervention. Results: A total of 78 patients were evaluated during the study period: 69 (88.5%) received TIXA/CILGA as pre-exposure prophylaxis and 9 (11.5%) as a treatment strategy. A total of 52 (66.6%) patients were treated with standard chemotherapy at HO departments; 21 (27%) underwent HSCT, and 5 (6.4%) received CAR-T cell therapy. All children with COVID-19 receiving TIXA/CILGA presented a mild degree of severity. The most common clinical manifestations were fever, cough and coryza. At least one adverse event (AE) was reported in two (3.8%) patients excluding standard injection site reactions. Reported AEs were mild or moderate in intensity. One child reported mild myalgia and one reported moderate bone pain and weakness. Conclusions: In our observational multicenter cohort study, we explored the use of TIXA/CILGA as pre-exposure prophylaxis and treatment for COVID-19 among immunocompromised pediatric patients. While our findings suggest a potential benefit in preventing and managing COVID-19 in this vulnerable population, it is important to note the study’s non-comparative design. Our results highlight the need for well-designed clinical trials to confirm these observations and further assess the efficacy and safety of TIXA/CILGA in immunocompromised children.

Published
2024
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