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Background: The case reported presents a rare CD19− phenotype shift of acute lymphoblastic leukaemia clone during the course of relapse/refractory ALL in a paediatric patient. We explore possible reasons promoting CD19 negative cell selection, including discrete mutations and anti-CD19 treatment, which is gaining importance as targeted therapies such as blinatumomab enter standard treatment protocols. Case presentation: A 9-year-old male patient was admitted to the Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Poland with fatigue, anaemia and hepatosplenomegaly, and was subsequently diagnosed with B lymphocyte acute lymphoblastic leukaemia. Initial standard genetic analysis did not show significant chromosomal aberrations, and the patient underwent chemotherapy in line with the intermediate-risk protocol. After initially achieving remission, the disease relapsed, and the patient required hematopoietic stem cell transplantation (HSCT). In-depth retrospective microarray analysis performed at this point revealed additional risk factors including hyperdiploidy. After staying in remission for several months, a second recurrence was diagnosed which prompted targeted treatment application (Blinatumomab) and subsequent HSCT. The third leukemic relapse diagnosed shortly after second HSCT limited treatment options to last-resort CAR T cell therapy in Germany. Subsequent immunophenotyping revealed lack of CD19 expression by ALL clones and disqualified the patient from treatment. The patient died in October 2019 from disease progression. Conclusions: The case we report highlights the importance of in-depth molecular diagnostics and monitoring of relapse/recurrent ALL cases in order to identify and manage as many potential risk factors as possible during treatment. This gain importance as selective targeted treatments use increases, as antigenic phenotype and its changes directly influence the efficacy of such treatments, and therefore patients’ prognosis.

Infections are still a major reason of morbidity and one of the most common causes of death after hematopoietic cell transplantation (HCT). Antimicrobial prophylaxis plays a crucial role in decreasing non-relapse mortality after HCT. The objective of this guideline paper is presentation of current recommendations of antimicrobial prophylaxis for children and adults after hematopoietic cell transplantation, prepared in cooperation of Polish scientific hematological societies. Recommendations were prepared by the working group and finally approved by all 23 Polish transplant centers for children and adults. Existing ECIL (European Conference on Infections in Leukemia) and EBMT (European Society of Blood and Marrow Transplantation) guidelines, as well as results of survey performed among all Polish transplant centers, were the background material for working group. Recommendations are presented in sections dedicated to antibacterial prophylaxis, antifungal prophylaxis, antiviral prophylaxis, as well as prophylaxis of toxoplasmosis and infections with Pneumocystis jiroveci. Recommendations on principles of vaccination against COVID-19 are provided based on the state of knowledge in September 2021. A section on guidelines of environmental prophylaxis is also presented.

The aim was to evaluate the incidence, clinical course, and outcome of adenoviral infection (AdVI) in pediatric patients diagnosed and treated due to cancer and in pediatric recipients of hematopoietic stem cell. Over a 72-month period, all-in 5599 children with cancer: 2441 patients with hematological malignancy (HM) and 3158 with solid tumors (ST), and 971 patients after transplantation: 741 after allogeneic (allo-HSCT) and 230 after autologous (auto-HSCT) were enrolled into the study. Among cancer patients, 67 episodes of AdVI appeared in 63 (1.1%) children, including 45 (1.8%) with HM and 18 (0.6%; P < .001) with ST. Within transplanted patients, AdVIs were responsible for 88 episodes in 81 (8.3%) children (P < .001), including 78 (10.5%) patients after allo-HSCT and 3 (1.3%) after auto-HSCT. Time to develop AdVI was short, especially after allo-HSCT. The most common clinical manifestation in cancer patients was enteritis diagnosed in 63 (94.0%) cases, while among HSCT recipient asymptomatic adenoviremia was found in 36 (40.9%) cases and the most common clinical manifestation was urinary tract infection. Cancer patients with disseminated disease, as well as HSCT recipients with either asymptomatic viremia or disseminated disease, received antiviral treatment. The most commonly used first-line therapy was cidofovir. None of the cancer patients died due to AdVI, while within HSCT recipients three patients developed disseminated adenoviral disease and died despite antiviral treatment. In cancer patients, AdVIs are rare and associated with very good prognosis even without specific treatment. However, in allo-HSCT recipients, disseminated disease with fatal outcome is more likely to occur.

Krzysztof Czyzewski,1 Magdalena Dziedzic,1 Malgorzata Salamonowicz,2 Jowita Fraczkiewicz,2 Olga Zajac-Spychala,3 Agnieszka Zaucha-Prazmo,4 Jolanta Gozdzik,5 Przemyslaw Galazka,6 Natalia Bartoszewicz,1 Ewa Demidowicz,1 Jan Styczynski1 1Department of Pediatric Hematology and Oncology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Bydgoszcz, Poland; 2Department of Pediatric Transplantation, Oncology and Hematology, Medical University, Wroclaw, Poland; 3Department of Pediatric Oncology, Hematology and Transplantology, University of Medical Sciences, Poznan, Poland; 4Department of Pediatric Hematology, Oncology and Stem Cell Transplantation, Medical University, Lublin, Poland; 5Stem Cell Transplant Center, University Children’s Hospital, Department of Clinical Immunology and Transplantology, Jagiellonian University Collegium Medicum, Krakow, Poland; 6Department of General and Oncological Surgery for Children and Adolescents, Collegium Medicum, Nicolaus Copernicus University Torun, Bydgoszcz, PolandCorrespondence: Krzysztof CzyzewskiDepartment of Pediatric Hematology and Oncology, Collegium Medicum in Bydgoszcz, Nicolaus Copernicus University in Torun, Ul. Sklodowskiej-Curie 9, Bydgoszcz 85-094, PolandTel +48 52 585 48 60Fax +48 52-585 4087Email k.czyzewski@cm.umk.plObjective: The analysis of epidemiology, risk factors and outcome of viral infections in children and adolescents after hematopoietic cell transplantation (HCT).Methods: In this multicenter nationwide study a total of 971 HCT procedures (741 allo-HCT; 230 auto-HCT) over a period of 6 years were analyzed.Results: During this period 801 episodes of viral infections were diagnosed in 442 patients. The incidence of viral infections was 57.9% in allo-HCT and 4.8% in auto-HCT patients. The most frequent infections after allo-HCT were caused by cytomegalovirus (CMV), polyoma BK virus (BKV) and Epstein–Barr virus (EBV). The majority of infections occurred within the first 4 months after allo-HCT and over 80% required pharmacotherapy or symptomatic therapy. The median time of treatment of specific viral infection ranged from 7 (for EBV) to 24 (for CMV) days. The highest mortality was observed in case of CMV infection. The risk factors for viral infections were allo-HCT, acute leukemia, acute and chronic graft versus host disease (a/cGVHD), and matched unrelated donor (MUD)/mismatched unrelated donor (MMUD)-HCT. The risk factor for death from viral infection were CMV-IgG seropositivity in acute lymphoblastic leukemia recipient, and MUD/MMUD-HCT. The incidence of EBV infection requiring pre-emptive treatment with rituximab in allo-HCT children was 19.3%. In 30.8% cases of EBV infection, these episodes were preceded by other viral infection and treated with antivirals, which did not prevent development of EBV-DNA-emia with need of rituximab treatment in 81.5% cases. In 47.7% of these cases, GVHD was a factor enabling development of significant EBV-DNA-emia during antiviral therapy of other infection.Conclusion: We have shown that antiviral drugs do not prevent EBV reactivation in allo-HCT pediatric patients.Keywords: children, EBV, HCT, infectious complications, risk factors analysis, viral infections

The objective of the study was the analysis of incidence and outcome of invasive fungal disease (IFD) in children treated for malignancy (PHO, paediatric hematology-oncology) or undergoing hematopoietic cell transplantation (HCT) over a period of six consecutive years in nationwide study. A total number of 5628 patients with newly diagnosed malignancies and 971 patients after HCT (741 allo-HCT and 230 auto-HCT) were screened for infectious complications in biennial reports. IFD incidence was lower among PHO patients: 8.8% vs 21.2% (P .0001) and survival from IFD was better: 94.2% vs 84.1% (P .0001). Auto-HCT patients had lower incidence (10.9% vs 24.4%) and lower mortality than allo-HCT patients. Introduction of national antifungal prophylaxis programme in HCT and acute leukaemia patients decreased incidence of IFD in HCT (from 23.1% to 13.4%) and AML on conventional chemotherapy (from 36% to 23%) but not in ALL patients during chemotherapy. In multivariate analysis, the incidence of IFD was higher in patients after HCT, diagnosed for ALL, AML or NHL, and in patients 10 years old. Factors contributing to death with infection were as follows: undergoing HCT, diagnosis of acute leukaemia (ALL or AML) and duration of treatment of infection 21 days. In conclusion, the incidence of IFD in allo-HCT and in AML patients on chemotherapy has decreased after introduction of national programme of antifungal prophylaxis, while the incidence of IFD in ALL patients on chemotherapy did not change significantly. The outcome of IFD both in PHO and HCT patients has largely improved in comparison with historical international data.

Borowik Joanna, Kuśmierczuk Kinga, Brodowski Wojciech, Kozak Katarzyna, Jawoszek Patryk, Zaucha-Prażmo Agnieszka. Pseudohemangioma of the head - diagnostic difficulties in a pediatric patient with Ewing’s sarcoma of the scalp: case report. Journal of Education, Health and Sport. 2019;9(9):513-525. eISSN 2391-8306. DOI http://dx.doi.org/10.5281/zenodo.3417064 http://ojs.ukw.edu.pl/index.php/johs/article/view/7455 The journal has had 5 points in Ministry of Science and Higher Education parametric evaluation. § 8. 2) and § 12. 1. 2) 22.02.2019. © The Authors 2019; This article is published with open access at Licensee Open Journal Systems of Kazimierz Wielki University in Bydgoszcz, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 25.08.2019. Revised: 30.08.2019. Accepted: 14.09.2019. Pseudohemangioma of the head - diagnostic difficulties in a pediatric patient with Ewing’s sarcoma of the scalp: case report Borowik Joanna Urszula, joannaa.borowik@gmail.com, Students’ Research Group at the Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Kuśmierczuk Kinga, kinga.kusmierczuk@gmail.com, Students’ Research Group at the Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Brodowski Wojciech, brodowski.wojciech@gmail.com, Students’ Research Group at the Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Kozak Katarzyna Anna, katarzyna.kozak95@gmail.com, Students’ Research Group at the Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Jawoszek Patryk, patryk.jawoszek@gmail.com, Students’ Research Group at the Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Zaucha-Prażmo Agnieszka, agnieszkazauchaprazmo@umlub.pl, Department of Hematology, Oncology and Pediatric Transplantology, Medical University of Lublin, Profesor Antoni Gębala Street 6, 20-093 Lublin, Poland Abstract Introduction: Ewing’s sarcoma (ES) is the second most common malignant bone tumor of childhood and adolescence. Typically, ES affects the long bones, pelvis and the trunk. Primary location in the scalp is rare. Aim of study: The aim of our study is to focus on the case of a pediatric patient with Ewing's sarcoma located in the scalp, which adopted a cavernous angioma mask, and to assess the available knowledge on the diagnosis and treatment of the extracostal ES located in the scalp. Material and methods: The research method was a case study. The research material was the patient's medical record, and the technique used was an analysis. An unsystematic review of Polish and English-language scientific literature was conducted. Electronic databases: PubMed, SCOPUS and Google Scholar were searched using the keywords: sarcoma, Ewing, hemangioma, pseudohemangioma, the head, the scalp. Results: When the girl was 6 months old, a tubercle appeared on her head. It was asymptomatic, but gradually increased. Imaging studies suggested the diagnosis of cavernous hemangioma. The tumor did not respond to treatment and still was growing. A decision was made to include resection. The upward tumor underwent histopathology with immunohistochemical assessment and genetic testing. The result pointed to extraskeletal Ewing’s sarcoma. Conclusions: Despite the few cases described in the literature, remember about atypical locations of primary Ewing sarcoma such as scalp. Making the right diagnosis was a challenge for the multi-specialist team of doctors. Histopathological examination supplemented by immunohistochemical assessment and genetic testing gave a definitive diagnosis and enabled proper treatment. Key words: sarcoma; Ewing; hemangioma; pseudohemangioma; the head; the scalp

Jedlińska Dorota, Kaleta Marcelina, Zawitkowska Joanna, Kościuk Andrzej, Lejman Monika, ZauchaPrażmo Agnieszka, Drabko Katarzyna. Multiple complications of the induction phase chemotherapy for childhood acute lymphoblastic leukemia. Journal of Education, Health and Sport. 2019;9(8):121-126. eISNN 2391-8306. DOI http://dx.doi.org/10.5281/zenodo.3371094 http://ojs.ukw.edu.pl/index.php/johs/article/view/7267 The journal has had 5 points in Ministry of Science and Higher Education parametric evaluation. § 8. 2) and § 12. 1. 2) 22.02.2019. © The Authors 2019; This article is published with open access at Licensee Open Journal Systems of Kazimierz Wielki University in Bydgoszcz, Poland Open Access. This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the original author (s) and source are credited. This is an open access article licensed under the terms of the Creative Commons Attribution Non commercial license Share alike. (http://creativecommons.org/licenses/by-nc-sa/4.0/) which permits unrestricted, non commercial use, distribution and reproduction in any medium, provided the work is properly cited. The authors declare that there is no conflict of interests regarding the publication of this paper. Received: 05.07.2019. Revised: 25.07.2019. Accepted: 19.08.2019. Multiple complications of the induction phase chemotherapy for childhood acute lymphoblastic leukemia Dorota Jedlińska1, Marcelina Kaleta1, Joanna Zawitkowska1, Andrzej Kościuk1, Monika Lejman2, Agnieszka Zaucha-Prażmo1, Katarzyna Drabko1 1Department of Pediatric Hematology, Oncology and Transplantology, Medical University of Lublin, Poland 2Department of Pediatric Hematology, Oncology anf Transplantology, University Children’s Hospital, Genetic Diagnostic Laboratory, Lublin, Poland Corresponding author: Dorota Jedlińska, MD, Department of Pediatric Hematology, Oncology and Transplantology, University Children’s Hospital, Antoniego Gębali Street 9, 20-093 Lublin, Poland Email: dorkajed@gmail.com ABSTRACT Chemotherapy for childhood acute lymphoblastic leukemia (ALL) despite of high efficiency often leads to many different complications, what bring consequences like therapy failure, death, breaks in chemotherapy, elongate hospitalization. We reported case old 6-year-old girl treated with ALL, who experienced many, severe complications of chemotherapy during the induction phase of treatment. Key words: complication, chemotherapy, acute lymphoblastic leukemia, children

IntroductionUltrasonography plays an important role in evaluation of many diseases in pediatric population. The noninvasiveness of the method allows to its wide use in children. It is a first-line diagnostic test for detecting lymphadenopathy, benign and malignant abnormalities of the thyroid gland, abdominal structures or testes in boys. The Ultrasound Screening Program “No to Cancer in Children” was introduced to detect neoplastic lesions and possible developmental disorders in children with no symptoms to allow early diagnosis.Material and methodsThe children were scanned in special mobile ambulance - Ronald McDonald Care Mobile, equipped with two high-tech ultrasound devices. Ultrasound scans, including cervical, abdominal, pelvical and scrotal ultrasound were performed in population of asymptomatic children. In the years 2006-2019, 67.594 children, 34.892 boys and 32.702 girls aged from 9 months to 6 years were examined.ResultsTotally, 18.544 various abnormalities were detected. In case of the neck they were found in 7542 children, which represent nearly 11.2% of all patients. Changes in abdominal ultrasound were found in 4.496 cases (6.65%). Abnormalities of the male reproductive system were detected in 6.474 boys (18.5%). Twelve tumors were detected.ConclusionsScreening has proved to be very useful for the early identification cancerous as well as precancerous lesions. It is also worth continuing for a reason of effective detection of other childhood anomalies.

Additional file 1. List of chemotherapy protocols.

Gut colonization with antibiotic-resistant bacteria (ARB) is associated with a significantly decreased overall survival in adult patients undergoing allo-HCT because of an increased treatment-related mortality.The objective of this multicenter study was the analysis of impact of gut colonization status and the use of antibiotics on development of gastro-intestinal (GI) graft-versus-host disease (GVHD) of allo-HCT in children.All consecutive patients who underwent allo-HCT over a period of three years in all pediatric HCT centers in Poland were analyzed for the impact of gut colonization on GI GVHD, with respect to standard of care including prophylaxis of infections and supportive therapy.At the time of allo-HCT, 44.2% of pediatric patients were colonized by ARB. Decontamination therapy with antibiotics was applied in 78% of children. Gut decontamination prophylactic therapy with antibiotics decreased the risk of acute GI GVHD. The use of gentamicin contributed to decreased rate of GVHD, while the use of ciprofloxacin and colistin contributed to increased incidence of GVHD after allo-HCT in children. Sepsis with ARB and non-MFD transplant contributed significantly to worse survival, while neither colonization nor gut decontamination had an impact on overall survival.Gut decontamination therapy contributed to lower incidence of acute GI GVHD in children undergoing allo-HCT, and the use of specific antibiotics might be responsible for this effect.

BK virus is one of the most common causes of hemorrhagic cystitis (HC) in children undergoing hematopoietic cell transplantation (HCT). Viruses can be found in urine and serum samples of immunocompromised patients. Malignant diseases, age, cell source, day of granulocyte reconstitution, conditioning regimen, or use of total body irradiation may play an important role in BKV epidemiology, development of hemorrhagic cystitis course, and outcome. The aim of this study was to evaluate the incidence, clinical course, and risk factors for BKV-HC in children undergoing HCT. A total number of 133 patients who were prospectively tested for BKV colonization/infection were enrolled into this multicenter analysis. Episodes of BKV-HC occurred in 36/133 (27%) enrolled subjects. In a univariate analysis for BKV-HC incidence, the following factors were significant: age >5 years, peripheral blood transplantation, matched unrelated donor (MUD) transplantation, busulfan-cyclophosphamide-melphalan conditioning regimen, and acute myeloblastic leukemia (AML) diagnosis. Presence of acute graft-versus-host disease (aGVHD) in liver and gut GVHD was a significant risk factor of BKV-HC. No BKV-attributed deaths were reported. In multivariate analysis, the incidence of HC was significantly higher in patients with AML, age >5 years, MUD transplants, and children with GVHD. HC is a frequent complication after HCT among children causes prolonged hospitalization but rarely contributes to death. We identified risk factors of BKV-HC development in children, with focus on aGVHD: we concluded that excessive immune reaction connected with GVHD and immunosuppression drugs might play a pivotal role in the development of BKV-HC.

Background/aim The risk factors, clinical features and non-hematological toxicity profiles during chemotherapy in acute lymphoblastic leukemia (ALL) patients treated in pediatric hematology centres were analysed. Materials and methods A total of 902/1872 children were reported as having grade 3 or 4 toxicity. Results Among the analysed toxicities, infection and gastrointestinal and liver toxicities were the most common. The median follow-up was 6.8 years. Overall survival and event-free survival rates for the analysed group were lower than those reported for the group without grade ≥3 toxicity. In univariate analysis, we identified the number of toxic episodes, the risk group and remission status that had a significant impact on the outcome. Multivariate analysis demonstrated the risk group and the number of toxic episodes ≥3 to be statistically significant for the results. Conclusion The toxic profiles investigated in our report should be used in future efforts to decrease the burden of side effects during chemotherapy.