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Therapy results in pediatric Hodgkin lymphoma reflect remarkable progress in pediatric oncology. In the last decade, relevant development of new therapeutic options for children with refractory or relapsed disease has been made. In this study, we retrospectively analyzed therapy results and risk factors in children treated in a single oncology center according to five therapeutic protocols. Data from 114 children treated by a single institution between 1997 and 2022 were analyzed. Classic Hodgkin lymphoma therapy results were divided into four therapeutic periods: 1997–2009, 2009–2014, 2014–2019, and 2019–2022. For nodular lymphocyte-predominant Hodgkin lymphoma, data from one therapeutic protocol was analyzed. For the entire group, the 5-year probability of overall survival was 93.5%. There were no statistically significant differences between therapeutic periods. The occurrence of B symptoms at diagnosis and incidence of relapse were risk factors for death (p = 0.018 and p p = 0.086). The 5-year probability of event-free survival for all patients was 91.3%. Five patients died, and the most common cause of death was relapse. Modern therapeutic protocols in pediatric Hodgkin lymphoma are marked by excellent outcomes. Patients with disease relapses have a notably high risk of death, and the development of new therapeutic options for this group remains one of the main goals of current trials.

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.

Introduction: We analyzed the epidemiology and outcomes of treatment of invasive fungal disease (IFD) in children during anticancer therapy (PHO, pediatric hematology and oncology) or after hematopoietic cell transplantation (HCT) over a period of eight consecutive years in a single-center study. Material and methods: Overall, a total of 254 HCTs were performed, and 415 children were newly diagnosed for malignancy. Incidence, epidemiology and outcome of IFD were analyzed. Results: The cumulative incidence of any IFD was 32.6% in allo-HCT, 22.2% in PHO, and 6.0% in auto-HCT patients. The incidence of proven +probable IFD was 12.6%, 10.4%, and 6.0%, respectively. As many as 77.0% HCT and 67.4% PHO of fungal episodes occurred in acute leukemia patients: the highest incidence of any IFD was observed for acute lymphoblastic leukemia (29.3% in HCT; 40.5% in PHO) and for acute myeloblastic leukemia (51.1% in HCT; 65.0% in PHO) patients. There were no significant differences in the incidence of fungal infections in both allo-HCT and PHO patients between the 2-year periods. Factors contributing to an increased risk of IFD in allo-HCT patients were: CMV replication, and acute and chronic graft-versus-host disease (GvHD). Survival from IFD was 91.9% in PHO, and 78.1% in HCT patients. Fungal pneumonia in HCT patients resolved in 62.9%, while in PHO patients it resolved in 93.5%. Conclusions: The risk of IFD in allo-HCT patients is much higher than in auto-HSCT and PHO patients. The outcome of IFD is better in PHO and auto-HCT than in allo-HCT settings.

The aim of this multi-center study was to evaluate the incidence, clinical course, and risk factors for bacterial multidrug-resistant (MDR) gastrointestinal tract infections (GTI) among children undergoing allogeneic and autologous hematopoietic cell transplantation. A total number of 175 pediatric patients (aged 1–18 years), transplanted between January 2018 and December 2019, who were tested for bacterial colonization/infection were enrolled into this multi-center analysis. Episodes of MDR GTI occurred in 77/175 (44%) patients. In multivariate analysis for higher GTI incidence, the following factors were significant: matched-unrelated donor (MUD) transplantation, HLA mismatch, presence of graft-versus-host disease (GVHD), and gut GVHD. The most common GTI were Clostridium difficile (CDI), multidrug-resistant Enterobacteriaceae (Klebsiella pneumoniae, Escherichia coli extended-spectrum β-lactamase), and Enterococcus HLAR (high-level aminoglycoside-resistant). No MDR GTI–attributed deaths were reported. MDR GTI is a frequent complication after HCT among children, causes prolonged hospitalization, but rarely contributes to death. We identified risk factors of MDR GTI development in children, with focus on GVHD and unrelated donor and HLA mismatch. We conclude that the presence of Clostridiales plays an important anti-inflammatory homeostatic role and decreases incidence of GVHD or alleviate its course.

Introduction: Letermovir (LMV) is a new antiviral agent approved in 2017 for prophylaxis to prevent cytomegalovirus (CMV) reactivation in CMV-seropositive allogeneic hematopoietic cell transplant recipients. Numerous reports on real-world experiences with LMV have been presented at international hematology and hematopoietic cell transplantation meetings. The objective of this study was to summarize data reported 2019–2020 on primary prophylaxis with LMV in adult patients. Methods: We analyzed 19 studies published or presented in 2019 and 2020, including two studies presented twice. Results: An overall 817 patients received primary prophylaxis with LMV. In 12 studies with a control group, the rate of breakthrough infection was 99/577 (17.2%) vs. 874/1,525 (57.3%), odds ratio (OR) =6.5 [95% confidence interval (CI) =5.1–8.2), p

Introduction: Nowadays, a significant number of children with acute leukemia can be cured. Iron overload, related to blood transfusions and its long-term complications, remains a problem. Elevated ferritin concentration is often observed in this group. The aim of this study was to evaluate the prognostic value of serum ferritin on long-term outcomes in children treated for acute leukemia. Material: We studied 71 patients treated for acute lymphoblastic (ALL) or myeloblastic (AML) leukemia between 2005 and 2011. Serum ferritin concentration, serum transaminases activity, lactic dehydrogenase and C-reactive protein levels (CRP) were analysed. Serum ferritin >1,000 µg/L was considered to be a marker of iron overload. Results: Thirty-seven patients (52.1%) had iron overload. Ferritin serum concentration correlated with alanine aminotranferase activity ( p =0.001) and CRP concentration ( p =0.012). A total of 19 (26.76%) patients died during follow-up. Ferritin level was higher in patients with AML vs. ALL. There was a significant difference in long-term outcomes with respect to high ferritin concentrations, both in patients undergoing haematopoietic cell transplantation (HCT) and in the non-HCT group. Conclusions: In both groups, patients with higher ferritin concentrations had worse overall and event-free survivals and a higher relapse incidence. Ferritin concentration >1,000 µg/L was the strongest determinant of long-term treatment outcome. Ferritin serum concentration >1,000 µg/L is an adverse prognostic marker of survival in children with acute leukemia treated with chemotherapy with or without HCT.

Background/Aim: Comparison of transplant outcomes in long-term follow-up of children after total body irradiation (TBI)- or chemotherapy-based conditioning allogeneic hematopoietic cell transplantation (allo-HCT). Patients and Methods: Patients undergoing allo-HCT for Acute lymphoblastic leukemia (ALL) conditioned either with TBI (n=55) or chemotherapy (n=84) were compared. The following transplant outcomes were analyzed: overall survival (OS), event-free survival (EFS), relapse incidence (RI), and graft-versus-host-disease (GVHD)-free-relapse-free survival (GRFS). Results: All analyzed long-term transplant outcomes were significantly better for patients conditioned with TBI at 2 years after transplant. OS at 2 years was 84% after TBI and 60.5% after chemotherapy-conditioning (p=0.005). Risk factor analysis showed that two factors, TBI-based conditioning and transplant in first remission of ALL, significantly improved OS, EFS, GRFS, and decreased RI. Conclusion: TBI-based conditioning before allogeneic HCT in children with acute lymphoblastic leukemia provides significantly better transplant outcomes, when compared to chemotherapy-based conditioning.

Background: Infectious complications (IC) caused by bacterial strains often impede anticancer therapy. The study aimed to retrospectively analyze bacterial IC that could help predict the risk and o...

BackgroundThrombopoietin receptor agonists have been repeatedly confirmed to be safe, efficient, and well tolerated in pediatric patients with chronic immune thrombocytopenia (cITP).Material and methodsIn this report, we present data summarizing the Polish experience of the use of eltrombopag in cITP patients, refractory to standard first-line care. Our analysis was based on clinical and epidemiological data from the Nationwide Therapeutic Program 2018–2020. Quality of the response to the eltrombopag treatment was defined according to the International Consensus Guidelines as follows: complete response (CR) defined as platelet count (PLT) ≥100 × 109/L and absence of bleeding; response (R) defined as PLT ≥30 × 109/L and at least two-fold increase in the baseline count and absence of bleeding.ResultsWe evaluated 60 patients (33 boys and 27 girls) with chronic and refractory ITP. Median age at beginning of treatment was 9.5 years. Median PLT at the first eltrombopag administration was 30 × 109/L. The median follow-up was 7 months (range, 3–22 months). After 1 week of treatment, response (R) was noted in 53.3% (95% confidence interval [CI]: 40.7%–66.0%) patients, and complete response (CR) was seen in 21.6% (95% CI: 11.2%–32.1%). We evaluated the long-term duration of the response and found that it was obtained in 84.4% (95% CI: 71.8%–97.0%) and 88.9% (95% CI: 77.0%–100%) of patients after 6 and 12 months, respectively, of eltrombopag therapy, while CR was reached, respectively, in 46.9% (95% CI: 29.6%–64.2%) and 29.6% (95% CI: 12.4%–46.9%) patients. No serious adverse events were reported.ConclusionOur data support the safety and efficacy of eltrombopag use in cITP pediatric patients.

Background Oral mucositis (OM) is considered to be one of the worst and most debilitating complications of conditioning for hematopoietic cell transplantation (HCT). Prevention and treatment of this complication is one of the utmost priorities of supportive therapy during transplant procedure. The objective of this study was the analysis of the influence of palifermin, keratinocyte growth factor (KGF), on transplant outcomes in patients undergoing allo-HCT. Patients and methods A total of 253 allo-HCTs performed between 2003-2018 in patients aged 0-19 years at a single center were analyzed. KGF was administered in 161 HCTs. Uni- and multivariate risk factor analyses were performed. Results In spite of reducing the duration and grade of mucositis, no prognostic impact of KGF was shown for overall survival, event-free survival, relapse incidence, acute and chronic graft-versus-host disease (GVHD), nor GVHD-free relapse-free survival. Conclusion Palifermin had no impact on transplant outcomes in children and adolescents undergoing allo-HCT.

IntroductionLeukemia belong to 31% of all childhood malignancies. Acute lymphoblastic leukemia (ALL) is the most frequent type of pediatric leukemia accounting for 80–85% of all cases. Progress in diagnostics and therapy of leukemia is dependent on international cooperation. The objective of the study was the analysis of non-hematological toxicity during intensive chemotherapy according to two consecutive intercontinental protocols.Patients and methodsA total number of 210 children diagnosed for ALL who were treated in single center between 2002 and 2018 were divided in two groups defined by therapeutic protocol: ALL IC-BFM 2002 (group 1) and ALL IC-BFM 2009 (group 2). Data were entered prospectively from 2002 into international ALL IC-BFM 2002 and ALL IC-BFM 2009 registry. Non-hematological toxicity was analyzed according to the criteria followed in protocols, compatible with CTCAE criteria.ResultsThe most frequent toxicities included hepatic toxicity with transaminitis and hyperbilirubinemia, infections, oral mucositis and gut toxicity with vomiting, and/or diarrhea. Non-hematological toxicity episodes calculated as a ratio per patient were comparably often observed in both the groups; however, the distribution was different. There were more grade III and less grade II toxicities. This was mainly related to significant increase in the rates of infections and transaminitis. However, there was a significant decrease in vomiting and central and peripheral neurotoxicity.ConclusionsIntensive treatment of ALL is burdened with frequent severe toxic and infectious complications. Further progress in therapy of pediatric ALL is dependent on sophisticated supportive therapy and very well experienced and knowledgeable therapeutic team.

Hematopoietic cell transplantation (HCT) is widely used as a treatment for acquired and congenital disorders. In recent years, a significant increase in transplant activity around the world has been observed, especially in Eastern European countries. This article aimed to assess progress and trends in pediatric HCT in Central-Eastern European countries between 2013 and 2018. Transplant activity survey in 2013 and 2018 in nine Central-Eastern European countries (Czech Republic, Croatia, Hungary, Lithuania, Poland, Romania, Slovakia, Slovenia, and Ukraine) was performed. The highest transplant rates in total were found in the Czech Republic and Hungary. When calculated per 10 million of the pediatric population, a 25.9% increase in the number of allo-HCT was observed with the highest in Croatia, Romania, Lithuania, and Poland; and a 12.2% increase in the number of auto-HCT was observed with the highest in Slovenia, Slovakia, Romania, Poland, Ukraine, and Croatia. We have shown, over the years 2013 and 2018, in some countries of Central-Eastern Europe that there was a significant increase in transplant activity, especially in those with the lower rates. This increase was observed mainly in centers already existing in 2013, especially in the allo-HCT setting. The rise of activity was significantly less influenced by the creation of new transplant centers or the increase in the number of pediatric transplant beds. In conclusion, our analysis indicates that in the Czech Republic, Hungary, Lithuania, and Slovenia, the actual infrastructure and the number of HCTs cover the needs, whereas in other countries, especially in Romania and Ukraine, the number of HCT needs to be increased.

IntroductionOral mucositis is regarded by patients as one of the worst and debilitating complications of conditioning and hematopoietic cell transplantation (HCT). Prevention of mucositis is one of the priorities of supportive therapy during and after conditioning.ObjectivesThe primary objective of the study was the analysis of efficacy of keratinocyte growth factor (KGF, palifermin) used in prophylaxis of oral mucositis in patients undergoing allo-HCT. The secondary objectives of the study included the analysis of the influence of palifermin on clinical course of oral mucositis and early transplant outcomes, as well as analysis of the contraindications of palifermin in patients undergoing allo-HCT.Patients and methodsA total number of 253 allo-HCT performed between 2003 and 2018 in patients aged 0–19 years in a single center were analyzed. Overall, in 161 HCTs, palifermin was administered.ResultsPatients receiving KGF were transplanted earlier in the context of calendar year, and more often received ATG, mainly due to the higher rate of unrelated donor transplants. Allo-HCT patients who were administered palifermin had shorter time of mucositis (median: 9 vs. 13 days, p < 0.001), lower mucositis grade (median: 2° vs. 3°; p < 0.001), shorter period of total parenteral nutrition (median: 19 vs. 22 days; p = 0.018), and lower incidence of episodes of febrile neutropenia (median: 39.1% vs. 83.1%; p < 0.001).ConclusionsThe use of palifermin has decreased duration and severity of oral mucositis in children after allo-HCT. Palifermin is a safe and well-tolerated compound in children undergoing allo-HCT.

No epidemiological data exist so far on invasive mucormycosis (IM) in Polish hematopoietic cell transplantation (HCT) and pediatric hemato-oncology (PHO) centers. The objective of this study was to analyze the incidence, clinical course, therapy, and outcome of IM in pediatric and adult patients undergoing HCT and children with hemato-oncological diseases in Poland. A total number of 12425 at-risk patients were retrospectively analyzed, and the period between 2010 and 2019 was included. Patients were analyzed in three groups: nontransplant children with malignancies, children undergoing HCT, and adults after HCT. Twenty-one patients were diagnosed with IM, including 15 children (10 non-HCT, 5 HCT) and 6 HCT adults. Proven IM was confirmed in 18 patients, probable in 2 patients, and possible in 1 patient. Proven IM was diagnosed in 7.1% of all patients with invasive fungal diseases (IFDs), including 8.1% among PHO patients, 5.4% among pediatric HCT patients, and 7.0% among adult HCT patients. Clinically, pneumonia was diagnosed in 10 (47.6%) patients, involvement of the paranasal sinuses was found in 3 (14.3%) patients, gastrointestinal disease was noted in 2 (9.5%) patients, and disseminated mucormycosis was found in 6 (28.6%) patients. The probability of overall survival in IM patients was 0.50 ± 0.11. Infection-related mortality (IRM) was 10% for pediatric nontransplant IM patients and 82% for transplant IM (pediatric + adult) patients (p = 0.004). Among the transplant patients, all adults died within 120 days. IRM for pediatric HCT patients was 60% (p = 0.038). The only prognostic factor was HCT, which adversely influenced survival in IM patients.

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

Background/aim The objective of the study was to propose clinical guidelines for the use of minimally invasive surgery (MIS) in pediatric oncology. Patients and methods Two groups of experts, including pediatric surgeons and pediatric oncologists were created in order to establish strategies of diagnostic and therapeutic surgical management in pediatric oncology. Results On the basis of the analysis of the existing literature, we elaborated guidelines that were graded according to the simple practical clinical system: yes/no. This project was dedicated to the following topics: adrenal tumors including neuroblastoma, renal tumors including Wilms tumor (nephroblastoma), ovarian tumors and pulmonary nodules and metastases (osteosarcoma). Conclusion Although existing data do not allow the recommendation of the use of MIS for all indications, this technique should currently be regarded as a standard of care in several areas of pediatric oncology.

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.

Background A retrospective analysis was performed to investigate the survival outcomes in pediatric acute lymphoblastic leukemia (ALL) based on time period. We hypothesized that improvement has been obtained with the time-dependent therapeutic era and rise in the gross domestic product (GDP) and Human Development Index (HDI). Materials and methods Data from 710 children who were treated for ALL between 1958 and 2018 at a single pediatric center were analyzed for probability of 5-year overall survival (pOS), event-free survival (pEFS) and relapse risk (pRR). Time periods were defined by the treatment protocols used in seven consecutive therapeutic eras. Results Over the 60-year period analyzed, pOS increased from 1.2% to 90.7%, pEFS from 1.2% to 86.6%, and pRR decreased from 98.8% to 9.9% for patients treated in the past decade. Risk of mortality for patients who received chemotherapy and hematopoietic cell transplant was reduced to 9.9% in the recent era, however, no statistically significant survival difference was found between patients treated with stem cell transplant and those not. Conclusion The therapeutic era, related to improved GDP and HDI, was a statistically significant predictor of increased OS from ALL.

Patients with Nijmegen breakage syndrome (NBS) can develop life-threatening immunodeficiency, which should be treated with hematopoietic stem cell transplantation (HSCT). We report the case of a 14-year-old girl with NBS who due to an increasing number of severe complications was referred for HSCT from a matched unrelated donor. After reduced-intensity conditioning and transplantation of peripheral blood hematopoietic cells, during the early post-transplant period (days 0-30), the girl suffered from severe mucositis, fever episodes, mild acute renal injury and facial vasculitis. All these complications were managed successfully. During the intermediate post-transplant period (days 30-100) a number of hepatic and gastrointestinal complications occurred, including cholecystitis, cholelithiasis with choledocholithiasis, pancreatitis as well as acute bleeding from the lower gastrointestinal tract caused by rectal and recto-sigmoid junction ulcers. All the obstacles were obviously attributable both to the primary congenital disease, its complications, and transplantation itself. We overcame these complications and treated the patient with the best possible and safe methods. The multidisciplinary approach based on combined surgical, endoscopic and conservative management of multiple post-transplant complications was successful for the patient.

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.

W artykule przedstawiono próbę rekonstrukcji przegrody chórowej z poł. XIII w. oraz lektorium z końca XIV w. w kościele Dominikanów w Krakowie w oparciu o wykopaliska, badania architektoniczne, źródła pisane i pomiar kościoła metodą skanowania laserowego. Pierwsza przegroda była ścianą działową przeprutą dwoma przejściami. Druga, w typie lektorium halowego, była konstrukcją pięcioprzęsłową z bocznymi przęsłami funkcjonującymi jako samodzielne kaplice, i ze środkowym przęsłem komunikującym nawę z chórem. W 1543 r. lektorium przebudowano w związku z urządzeniem na jego piętrze kaplicy św. Jacka. W l. 1581-83 kaplicę powiększono burząc lektorium.

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: Infections constitute a major problem for patients during oncological treatment or undergoing hematopoietic stem cell transplantation (HCT). Objective: The aim of the study was to analyze the epidemiology of viral infections in children during anticancer therapy (PHO, pediatric haematology and oncology) or after HCT over a period of consecutive 6 years in a single-centre study. Patients and methods: During this period, a total number of 182 HCTs were performed, and 306 children were newly diagnosed for malignancy. Incidence, hazard risk and outcome of infections were analyzed. Results: The cumulative incidence of viral infections was 61.7% in allo-HCT, 8.5% in PHO, and 4.1% in auto-HCT patients. The overall risk of viral infection in HCT patients was 17.3-fold higher (p < 0.0001) than in PHO patients. The risk was 30-fold higher for CMV and 63-fold higher for EBV, while the risk was comparable for influenza and adenovirus infection. Infections with polyoma BKV occurred only in HCT patients after allo-HCT. Factors contributing to increased risk of viral infections in allo-HCT patients both in uni- and multivariate analysis were: male sex, diagnosis of acute leukemia, alternative donor, CMV positive serostatus in recipient and/or donor, acute and chronic GVHD. All patients except two allo-HCT children survived viral infections. The cause of death were influenza and EBV-PTLD. Conclusions: The risk of viral infections in allo-HCT patients is much higher than in auto-HSCT and PHO patients, while the outcome of infections was better in the PHO and auto-HCT setting.

Background/aim Immune recovery is a key factor in the management of patients after allogeneic hematopoietic stem cell transplantation (allo-HSCT). This study analyzed the factors contributing to immune reconstitution after allo-HSCT. Patients and methods Overall, 65 children with malignant or non-malignant diseases were included in multivariate analyses. Results The following factors contributed to a faster immune recovery: peripheral blood as a stem cell source and reactivation of CMV infection for CD3+ and CD4+ lymphocyte subpopulations; reactivation of CMV infection for CD8+ subset; donor EBV-IgG+ and no EBV reactivation for CD19 lymphocytes; recipient age below 10 years and peripheral blood as a stem cell source for NK cells. For CD2 and CD4/CD8 ratio no factor was significant in multivariate analysis. Conclusion Patients receiving a graft from an EBV-IgG-positive donor and not having early EBV post-transplant viremia show faster recovery of the B-cells, while patients with early CMV-DNA-emia have a better re-establishment of T-cell subsets.

Aim The aim of the study was to evaluate immune reconstitution before making decisions of revaccination for patients after haematopoietic stem cell transplantation (HSCT). The reasons for the derogations from our algorithm were also analyzed. Patients The study included 81 patients aged 1.5 to 25.2 years, with 31 women and 50 men, and 55 allo-HSCT and 26 auto-HSCT patients. We analyzed time and degree of immunological reconstitution after HSCT and decisions that have been made with respect to revaccination, its postponement or implementation of immunoglobulin substitution. Results Time between HSCT and immunological assessment ranged from 5.1 to 27.4 months (median 11 months, including 8.6 months after auto-HSCT and 11.5 months after allo-HSCT). For patients for whom auto-HSCT was the last phase of oncological treatment, this median time was 6.7 months. Evaluation of immune system up to 8 months after HSCT was performed for 15/81 (18.5%) patients. Among the remaining 66 patients, the most common cause of delay was immunosuppression (22.7%), and in 16/66 (24.2%) cases, no cause was found in the medical records. After first evaluation of the immune system, 64/81 (79%) patients were advised to resume vaccination. Conclusions Immunological reconstitution analysis after HSCT was delayed in 81.5% of patients. In order to improve the effectiveness of the post-HSCT vaccination program, it seems appropriate to accelerate the first immunological evaluation and to differentiate the recommendations according to the type of transplantation and post-HSCT therapy, i.e. 3–6 months after auto-HSCT or last phase of oncological treatment and 6-12 months after allo-HSCT.

Introduction Nonneoplasmatic pineal cyst changes are quite often found during brain MRI of pediatric patients. Usually, they are asymptomatic; however, clinical signs such as headache, sight disorders, dizziness or hydrocephalia may occur. Objective Epidemiological and clinical evaluation of the occurrence of pineal cysts in children treated in a single oncological ward over a period of 7 years. Methods Retrospective analysis of the patients hospitalized in the Department of Pediatrics, Hematology and Oncology in Bydgoszcz and diagnosed for pineal cyst by MRI. Results The incidence of pineal cysts in patients who underwent MRI for various reasons was 5.2%. In 52% of the patients, MRI examination was performed to check their reported headache. The size of the cysts in MRI varied from 3 mm to 21 mm. Control MRI was performed in 64% of the children after 1–12 months. In 2 of them, little reduction of the size of the cyst was observed; in one case, slight enlargement was observed, and in the remaining, the size was stable. None of the patients required neurosurgical intervention. Conclusions Pineal cysts were diagnosed in 5.2% of the children with central nervous system disorder symptoms. In half of the cases, headache was the reason for starting the diagnostic procedures. Size of the pineal cysts observed in the population of pediatric patients usually does not exceed 10 mm. Most of the pineal cysts have mild clinical character but always require excluding the presence of malignant process. No clinical progression was observed within 6–12 month observation in any of the patients.

Varicella-zoster virus (VZV) infection in pediatric hemato-oncology patients can be a therapeutic problem when children are exposed to immunosuppression. The aim of this study is to evaluate the incidence of VZV infection, antiviral therapy and outcome in children with ALL treated in polish hemato-oncological centers between 2012 and 2019 years. This study included medical records of 1874 patients, aged 1 to 18 years, with newly diagnosed acute lymphoblastic leukemia. During chemotherapy, 406 children out of 1874 (21.6%) experienced viral infections. The incidence of VZV infection in the whole group children with ALL was 1.8%. Among them, 34 (8.4%) patients were diagnosed with VZV infection. Thirty-five episodes of viral infections were identified. The median time of VCV therapy was 12 days. Herpes zoster infection occurred in 24 (70.6%) children, and varicella in 10 (29.4%) ones. The average time from the start of chemotherapy to the appearance of herpes zoster was 7.26 ± 4.05 months. VZV infection occurred mainly during the maintenance therapy, the reinduction and induction phases. There was no correlation between steroid dosage or type and subsequent zoster. The total lymphocyte count of these patients on the first day of zoster was reduced. No serious complications were observed due to this infection. All patients survived. In conclusion, a low incidence of VZV infection was observed among pediatric patients with ALL in Poland. This analysis indicates that currently used therapeutic methods are effective in children with cancer and VZV infection. The main focus should be on the prevention of delayed chemotherapy.