Publications by authors named "Jan Styczynski"

This survey reports on the current practice and management of hemorrhagic cystitis (HC) among the EBMT centers. The survey response rate was 27%. The diagnostic search of BK polyomavirus (BKPyV) was available in 93% of centers and performed in urine (100%), blood/plasma (51%), and serum (12%).

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: spp. represent the most prevalent Gram-positive organisms in children with malignancies or undergoing haematopoietic cell transplantation (HCT), contributing to significant morbidity and mortality. This study aimed to assess the epidemiology, risk factors, treatment strategies, and outcomes of staphylococcal infections (SIs) in paediatric haemato-oncology (PHO) and HCT patients in Poland over a 12-year period.

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Background: Acute leukemia (AL) is the most prevalent pediatric malignancy and is frequently associated with systemic iron dysregulation, often leading to iron overload. This study aimed to characterize the regulatory mechanisms of iron metabolism in children with AL, considering treatment stages and associated clinical parameters.

Methods: A total of 149 children were stratified into four groups: newly diagnosed AL ( = 43), patients post-chemotherapy ( = 55), patients following hematopoietic cell transplantation (HCT; = 32), and healthy controls ( = 19).

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: , specifically , are becoming a great threat to hospitalized patients due to increasing antibiotic resistance. The aim of this study was to describe the epidemiology, clinical characteristics, antimicrobial susceptibility pattern and outcome of infections in pediatric cancer patients and hematopoietic stem cell transplant (HSCT) recipients in Poland. : A total of 125 episodes of species infections were reported in patients <18 years treated in Polish pediatric hematology and oncology centers over a period from 2012 to 2023.

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Scope: This position paper is intended for clinicians and data managers involved in the diagnosis, management, and reporting of bloodstream infection (BSI) and central venous catheter (CVC)-related BSI and CVC-related local skin/soft-tissue infections (lSSTI) in haematopoietic cell transplant (HCT) recipients.

Methods: The panel reviewed the relevant guidelines on BSI and CVC-related lSSTI definitions, and their applicability to HCT recipients. We developed practical recommendations aiming to establish their standardized reporting considering the unique features of HCT recipients.

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Neurofibromatosis type 1 (NF1) is a genetically determined disorder with a complex clinical picture, including disorders of many systems and organs, that predisposes to the development of various cancers. Approximately 10-30% of patients with NF1 develop plexiform neurofibromas (PN), non-malignant tumours growing along the nerves, tending to infiltrate nerves and nerve plexuses, and may grow into internal organs, the spinal canal, and middle ear, deforming bones and various regions of the body. Magnetic resonance imaging (MRI) plays a key role in the management of individuals during the course of PN, as a screening tool at baseline, for surveillance (in individuals with known PN), to evaluate treatment response, and for preoperative assessment for surgical planning.

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: (PSA) infections are associated with a high recurrence rate and high mortality in immuno-compromised patients. There are limited studies regarding pediatric hematopoietic cell transplantation recipients. : The nationwide multicenter study was conducted to analyze the epidemiology of PSA infections in children treated with chemotherapy (PHO, pediatric hematology and oncology) or undergoing hematopoietic allogeneic or autologous cell transplantation (HCT) in the period 2014-2023.

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Human herpes virus-6 (HHV-6) is the main cause of viral encephalitis in patients undergoing allogeneic hematopoietic cell transplantation (allo-HCT). From January 2005 to December 2014, 97 patients with HHV-6 encephalitis were reported in the EBMT registry. The incidence was 0.

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In the post-pandemic years, SARS-CoV-2 morbidity and mortality declined due to less pathogenic variants, active and passive immunization, and antiviral therapies. However, patients with hematological malignancies and/or undergoing hematopoietic cell transplantation (HCT) remain at increased risk for poor outcomes. Therefore, adherence to contact and droplet precautions is essential to avoid transmission, especially during epidemic waves.

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Among NF1-dependent tumors, the most common are optic pathway gliomas (OPGs). The objective of this study was the retrospective analysis of the course, indications for treatment, and effects of therapy for NF1-OPGs. We analyzed demographics, clinical and genetic data, imaging and ophthalmological parameters, their impact on therapeutic decisions, and the effectiveness of the therapy in 92 patients.

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: Over the past three decades, significant progress has been made in understanding the biology of neuroblastoma. The integration of prognostic factors has facilitated risk stratification and the development of targeted treatment approaches. This study aims to analyze the outcomes of pediatric patients with neuroblastoma treated at a single oncology center over a 30-year period.

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Background Chimeric Antigen Receptor T-cell (CAR-T) therapy targeting CD19 has transformed the treatment of relapsed/refractory B-cell precursor acute lymphoblastic leukemia (BCP-ALL). However, the phenotypic heterogeneity of CAR+ T-cells and their interactions with the immune system remain poorly understood. Here, we characterize CAR+ T-cell subsets and persistence in patients receiving standard-of-care tisagenlecleucel in an effort to optimize therapeutic efficacy in pediatric BCP-ALL.

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Background: The prognostic role of systemic inflammation markers in pediatric soft-tissue sarcomas (STS) remains unclear.

Procedure: This multicenter study investigated the prognostic significance of neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), lymphocyte-to-monocyte ratio (LMR), systemic immune-inflammation index (SII), systemic inflammatory response index (SIRI), C-reactive protein (CRP), and lactate dehydrogenase (LDH) in 213 pediatric patients diagnosed with STS in years 2002-2023. Patients were categorized into groups: rhabdomyosarcoma (RMS, n = 126), RMS-like (n = 57), and non-RMS (n = 30).

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Cytomegalovirus (CMV) remains the most important viral pathogen in patients after allogeneic haematopoietic cell transplantation (HCT), resulting in morbidity and mortality. The European Conference on Infections in Leukaemia (ECIL) brings together experts in several fields to produce evidence-based recommendations from comprehensive literature reviews. Management of CMV has been addressed twice before; the previous guideline update from the ECIL was published in 2019.

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To optimize and homogenize data on infectious diseases complications, the Infectious Diseases Working Party of the European Society for Blood and Marrow Transplantation (IDWP EBMT) participated in the 2023 EBMT international workshop focusing on the standardization of the definition of specific infections, including respiratory infections [1]. This is pertinent considering that a new software for data collection, including data on post-transplant infectious disease complications, was launched in 2023 and which will remain the main tool for future epidemiological studies. In this report, we briefly discuss our proposals for pneumonia definition, predominately focusing on bacterial and fungal pneumonias.

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Tuberculosis (TB) is rare following hematopoietic cell transplantation (HCT). In this multinational retrospective study, we report the frequency, characteristics, and outcome of TB following HCT performed during 2000-2019. Fifty-two patients (35 (67%) males, 15 (29%) children) from 24 centers developed TB following allogeneic (n = 47) or autologous (n = 5) HCT; with the relative frequency of 0.

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Purpose: Soon after the Russian invasion of Ukraine in February 2022, an initiative was organized to evacuate Ukrainian children with cancer, most initially to Poland. This study assessed the impact of this rapid increase in clinical need on the Polish system of pediatric cancer care.

Methods: This multicenter longitudinal approach was performed among all 19 Polish Pediatric Oncology Centers (PPOCs).

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Article Synopsis
  • Treosulfan is an important treatment in allogeneic haematopoietic stem cell transplantation (allo-HSCT) for children, offering lower toxicity than traditional drugs.
  • Researchers studied the effects of treosulfan on 83 pediatric patients to see how it impacts survival, graft failure, and graft-versus-host disease.
  • The results showed no significant correlation between treosulfan exposure and clinical outcomes or toxicity, indicating that standardized dosing is effective for pediatric allo-HSCT.
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Iron deficiency is one of the most common nutritional deficiencies worldwide and is the leading cause of anemia in the pediatric population (microcytic, hypochromic anemia due to iron deficiency). Moreover, untreated iron deficiency can lead to various systemic consequences and can disrupt the child's development. Therefore, a team of experts from the Polish Pediatric Society, the Polish Society of Pediatric Oncology and Hematology, the Polish Neonatology Society, and the Polish Society of Family Medicine, based on a review of the current literature, their own clinical experience, and critical discussion, has developed updated guidelines for the diagnosis, prevention, and treatment of iron deficiency in children from birth to 18 years of age.

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Background: A personalised approach to the treatment of acute myeloid leukemia (AML) in children and adolescents, as well as the development of supportive therapies, has significantly improved survival. Despite this, some patients still die before starting treatment or in an early phase of therapy before achieving remission. The study analysed the frequency, clinical features and risk factors for early deaths (ED) and treatment related deaths (TRD) of children and adolescents with AML.

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Article Synopsis
  • Pediatric patients with primary refractory or relapsed B-cell non-Hodgkin lymphoma (B-NHL) have a poor prognosis, as indicated by a study of 76 patients treated in Poland from 1995 to 2022.
  • The 5-year overall survival probability was 76.7% and event-free survival probability was 72.9%, but outcomes worsened for those with disease progression or relapse, particularly with a 0.0% survival rate for relapsing patients.
  • Key factors affecting prognosis included elevated lactate dehydrogenase, presence of B symptoms, and advanced disease at diagnosis, with hematopoietic stem cell transplantation showing potential as the most effective salvage therapy.
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