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Öğe A Case of Allergic Broncopulmonary Aspergillosis Associated With Hematopoietic Stem Cell Transplantation Due to Chronic Granulomatous Disease(Lippincott Williams & Wilkins, 2019) Malbora, Baris; Aksoylar, Serap; Ozdemir, Hamiyet H.; Ozdemir, Sinem; Kansoy, SavasAllergic bronchopulmonary aspergillosis is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus. This disorder is most commonly seen in patients with poorly controlled asthma and cystic fibrosis. It is rarely reported in chronic granulomatous disease patients; however, there are no cases reported with hematopoietic stem cell transplantation in the English literature. Herein, we report a patient with chronic granulomatous disease who had hematopoietic stem cell transplantation and subsequently developed allergic bronchopulmonary aspergillosis.Öğe COVID-19 disease in children and adolescents following allogeneic hematopoietic stem cell transplantation: A report from the Turkish pediatric bone marrow transplantation study group(Wiley, 2024) Bozkurt, Ceyhun; Hazar, Volkan; Malbora, Baris; Kupesiz, Alphan; Aygunes, Utku; Fisgin, Tunc; Karakukcu, MusaBackgroundData on the risk factors and outcomes for pediatric patients with SARS-CoV-2 infection (COVID-19) following hematopoietic stem cell transplantation (HSCT) are limited.ObjectivesThe 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.MethodIn this nationwide study, we retrospectively reviewed the data of 184 pediatric HSCT recipients who had COVID-19 between March 2020 and August 2022.ResultsThe median time from HSCT to COVID-19 infection was 209.0 days (IQR, 111.7-340.8; range, 0-3845 days). The most common clinical manifestation was fever (58.7%). While most patients (78.8%) had asymptomatic/mild disease, the disease severity was moderate in 9.2% and severe and critical in 4.4% and 7.6%, respectively. The overall mortality was 10.9% (n: 20). Deaths were attributable to COVID-19 in nine (4.9%) patients. Multivariate analysis revealed that lower respiratory tract disease (LRTD) (OR, 23.20, p: .001) and lymphopenia at diagnosis (OR, 5.21, p: .006) were risk factors for ICU admission and that HSCT from a mismatched donor (OR, 54.04, p: .028), multisystem inflammatory syndrome in children (MIS-C) (OR, 31.07, p: .003), and LRTD (OR, 10.11, p: .035) were associated with a higher risk for COVID-19-related mortality.ConclusionWhile COVID-19 is mostly asymptomatic or mild in pediatric transplant recipients, it can cause ICU admission in those with LRTD or lymphopenia at diagnosis and may be more fatal in those who are transplanted from a mismatched donor and those who develop MIS-C or LRTD. COVID-19 in children following HSCT is frequently asymptomatic/mild. Nonetheless, 12% of patients have such severe disease that they need intensive care. Adverse outcomes are expected in mismatched HSCT, lymphopenia, LRTD, and MIS-C.imageÖğe Hepatitis-Associated Aplastic Anemia: Etiology, Clinical Characteristics and Outcome(Lippincott Williams & Wilkins, 2022) Tufekci, Ozlem; Ozdemir, Hamiyet H.; Malbora, Baris; Ozbek, Namik Y.; Yarali, Nese; Erdem, Arzu; Evim, MelikeHepatitis-associated aplastic anemia (HAA) is a form of acquired aplastic anemia (AA) in which bone marrow failure develops after an acute attack of hepatitis. Bone marrow failure leading to AA is generally severe in cases of HAA and fatal if left untreated. This retrospective multicenter study investigated clinical and laboratory characteristics, possible causes, treatment, and outcome of HAA in children. Twenty patients from 8 centers were included in the study. Aspartate aminotransferase and alanine aminotransferase were <3 to 5xupper limit of normal (ULN) in 2 patients, <5 to 10xULN in 2 patients, and >10xULN in 16 patients. Acute liver failure developed in 5 (29%) patients. Pancytopenia was simultaneously present in 6 of 20 (30%) patients. Eleven of the 20 patients (55%) were alive, in remission and transfusion free. Those who were alive either had undergone hematopoietic stem cell transplantation and/or immunosuppressive treatment, except 1 patient who had received no treatment. Patients with the diagnosis of acute hepatitis should be evaluated and followed up carefully for presence of cytopenia, so that definitive treatment of AA can be initiated in a timely and appropriate manner when needed.Öğe Intracranial Bleeding in a Female Hemophilia Patient: Molecular Analysis of the Factor 8 Gene and Determination of a Novel Mutation(Galenos Yayincilik, 2018) Gunes, Burcak Tatli; Sivis, Zuhal Onder; Ataseven, Eda; Malbora, Baris; Turker, Meral; Belen, Fatma Burcu; Atabay, Berna; Atik, Tahir; Isik, Esra; Özkınay, FerdaÖğe A Mortal Complication in a Case with Mucopolysaccharidosis Type I Following Hematopoietic Stem Cell Transplantation: Pulmonary Haemorrhage(Dr Behcet Uz Cocuk Hastaliklari Ve Cerrahisi, 2021) Yazici, Havva; Canda, Ebru; Er, Esra; Malbora, Baris; Hismi, Burcu Ozturk; Onay, Huseyin; Aksoylar, SerapMucopolysaccharidosis type I (MPS I) is a lysosomal storage disease due to mutations within the gene IDUA encoding the alpha-L-iduronidase. The clinical manifestations concern multisystemic involvement. There are two disease modifying therapies, enzyme replacement therapy and haematopoietic stem cell transplantation (HSCT). Pulmonary haemorrhage (PH) is a rare complication of HSCT and the case was presented with the reason that the related reports were few in MPS I.
