Bone marrow mesenchymal stromal cells (BM-MSCs) are integral components of the bone marrow microenvironment, playing a crucial role in supporting hematopoiesis. Recent studies have investigated the potential involvement of BM-MSCs in the pathophysiology of acute lymphoblastic leukemia (ALL). However, the exact contribution of BM-MSCs to leukemia progression remains unclear because of conflicting findings and limited characterization.
View Article and Find Full Text PDFBackground: The management of pediatric acute myeloid leukemia (AML) is based on the prognostic risk classification of initial leukemia. Targeted next-generation sequencing (NGS) is a reliable method used to identify recurrently mutated genes of pediatric AML and associated prognosis.
Methods: In this study, we retrospectively evaluated the prognostic, and therapeutic utility of a targeted NGS panel covering twenty-five genes, in 21 children with de novo and 8 with relapsed or secondary AML.
This study retrospectively analyzed the outcomes of 61 pediatric patients with inborn errors of immunity (IEI) who underwent hematopoietic stem cell transplantation (HSCT) between 2011 and 2023. Patients were categorized into primary immunodeficiency disorders (PIDD), primary immune dysregulation disorders (PIRD), and congenital defects of phagocyte number or function (CDP). Median ages at diagnosis and HSCT were 9 and 30 months, respectively.
View Article and Find Full Text PDFBackground: Iron deficiency (ID) is the most common nutritional deficiency and cause of anemia in childhood. Iron deficiency anemia (IDA) is a common public health problem in Turkey as in the rest of the World. Asthma is a chronic inflammatory disease of the respiratory tract, affecting approximately 10% of the population in developing countries.
View Article and Find Full Text PDFHematopoietic stem cell transplantation is a curative therapy for pediatric patients with malignancies and blood or immune system disorders. However, recipients are at risk of opportunistic infections, including cytomegalovirus infection, due to immunosuppression from conditioning regimens. This retrospective study analyzed 439 pediatric hematopoietic stem cell transplantation patients to identify risk factors for cytomegalovirus infection and assess the impact of conditioning regimens.
View Article and Find Full Text PDFBackground/aim: The transfusion of blood products is a life-saving clinical practice in patients with bleeding, hemoglobinopathy, and cancer. It was aimed herein to analyze the frequency and types of blood component-related acute transfusion reactions (ATRs) in pediatric patients.
Materials And Methods: This retrospective study was conducted at a tertiary care academic pediatric hospital.
Pyruvate kinase (PK) is a key enzyme of anaerobic glycolysis. The genetic heterogeneity of PK deficiency (PKD) is high, and over 400 unique variants have been identified. Twenty-nine patients who had been diagnosed as PKD genetically in seven distinct paediatric haematology departments were evaluated.
View Article and Find Full Text PDFBackground: Data on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited.
Objectives: The study aimed to analyze the clinical signs, risk factors, and outcomes for ICU admission and mortality in a large pediatric cohort who underwent allogeneic HSCT prior to COVID-19 infection.
Method: In this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022.
Background: The published experience concerning autologous peripheral blood stem cell collection in children is very limited.
Methods: The data of pediatric patients who underwent autologous stem cell mobilization and apheresis between January 2011 and April 2020 were analyzed retrospectively.
Results: We studied retrospectively 64 mobilization and apheresis procedures in 48 pediatric patients (34 males, 14 females), mean age of 7.
Objective: This study aimed to assess the efficacy of different oral iron preparations prescribed for prevention of iron deficiency anemia in healthy infants.
Methods: This retrospective study enrolled infants aged between 6 and 12 months who were initiated on iron prophylaxis at four months of age. Enrolled children consistently used specific iron preparations (ferrous, ferric or liposomal iron) and had their complete blood counts and serum ferritin levels assessed within the 6-12 month timeframe.
Background: Herbaspirillum species are nonfermenting, aerobic, helical or curved, Gram-negative bacteria belonging to the class Betaproteobacteria, order Burkholderiales. To date, only a few studies have reported on the epidemiology, clinical symptoms, antibiotic susceptibility profiles, treatment and outcomes of Herbaspirillum huttiense -related infections in pediatric patients.
Methods: The aim of this study was to present 3 years of H.
The purpose of this study was to evaluate the association between interleukin-33 (IL-33) and its receptor Soluble Suppression of Tumorigenicity-2 (sST2) levels and bacterial infections during febrile neutropenia (FN) in pediatric patients with acute lymphoblastic leukemia (ALL). In this prospective, case-control study, participants were divided into 3 groups: ALL patients with FN (Group A), ALL patients without neutropenia and fever (Group B), and healthy children without infection and chronic disease (Group C). There were 30 cases in each group.
View Article and Find Full Text PDFBackground: We aimed to evaluate our pediatric HSCT recipients routinely monitored for adenoviremia and to determine the adequacy of this monitoring in predicting adenoviral disease (AD).
Methods: A retrospective cohort of patients who underwent allogeneic HSCT between January 2021 and August 2022, and routinely monitored for adenoviremia by real-time PCR was included in our survey. Demographic and clinical data of the patients were recorded.
Indian J Hematol Blood Transfus
January 2024
Unlabelled: Prophylaxis is the gold standard for the management of hemophilia A patients. It has been shown that prophylaxis regulated with pharmacokinetic (PK) data reduces frequency of bleeding and cost of treatment. To determine the best prophylaxis regimen, PK dosing tools using the Bayesian method have been developed.
View Article and Find Full Text PDFObjectives: To evaluate the effectiveness of national iron prophylaxis policy in 9-12 mo-old infants in Turkey.
Methods: This study was planned as a cross-sectional study, and it included healthy infants aged 9 to 12 mo who presented to the pediatric outpatient clinic for routine check-ups. Parents were interviewed to identify risk factors for iron deficiency (ID) and gather information on Fe - iron polymaltose complex (IPC) prophylaxis usage.
Anti-thymocyte globulin (ATG) has been successfully used for decades to prevent graft versus host disease before hematopoietic stem cell transplantation (HSCT) as a part of conditioning regimen. However, sometimes hypersensitivity reactions may limit its use. To evaluate hypersensitivity reactions experienced during rabbit-ATG infusion among children and present successful desensitization protocol.
View Article and Find Full Text PDFObjective: Beta thalassemia major is an inherited hemoglobin disorder resulting in chronic hemolytic anemia. Cardiac involvement is the main cause of death in patients. Speckle-tracking echocardiography is a feasible method for the evaluation of cardiac function via an assessment of the longitudinal deformation of the myocardium through the cardiac cycle.
View Article and Find Full Text PDFPurpose: It was aimed to evaluate the retinochoroidal microvascular alterations of pediatric beta-thalassemia patients and investigate the effect of blood transfusion on perfusion among transfusion-dependent thalassemia (TDT), by means of optical coherence tomography angiography (OCTA).
Methods: In this multicentered, prospective, cross-sectional study, 56 TDT, 14 non-TDT (NTDT), and 63 healthy children were evaluated. The vessel density (VD) in superficial capillary plexus (SCP), deep capillary plexus, radial peripapillary capillary network, choriocapillaris, and the foveal avascular zone area (FAZ) were evaluated by OCTA.
Objective: Patients with inherited bleeding disorders faced problems in accessing healthcare during coronavirus disease 2019 pandemic. This study aimed to investigate the health problems of patients with inherited bleeding disorders during the coronavirus disease 2019 pandemic.
Material And Methods: Children and adult patients with inherited bleeding disorders who had a coronavirus disease 2019 infection between March 2020 and November 2021 were retrospec- tively evaluated.
Background: In patients with acute lymphoblastic leukemia (ALL), the risk of thromboembolism increases due to hemostatic changes secondary to the primary disease and due to treatment-related factors. In this multicenter study, we aimed to research the frequency of central nervous system (CNS) thrombosis occurring during treatment, hereditary and acquired risk factors, clinical and laboratory features of patients with thrombosis, treatment approaches, and thrombosis-related mortality and morbidity rates in pediatric ALL patients.
Procedure: Pediatric patients who developed CNS thrombosis during ALL treatment from 2010 to 2021 were analyzed retrospectively in 25 different Pediatric Hematology Oncology centers in Türkiye.
Background: The aim of this study is to develop new perspectives to prevent or reduce potential organ damage due to iron-mediated oxidation in thalassemia major patients.
Methods: Seventy patients were included in this study. Blood samples were taken from the patients before and after transfusion.
Chediak-Higashi syndrome (CHS) is rare autosomal recessive disorder caused by bi-allelic variants in the Lysosomal Trafficking Regulator () gene. Diagnosis is established by the detection of pathogenic variants in in combination with clinical evidence of disease. Conventional molecular genetic testing of by genomic DNA (gDNA) Sanger sequencing detects the majority of pathogenic variants, but some remain undetected for several individuals clinically diagnosed with CHS.
View Article and Find Full Text PDFTo evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female.
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