Fanconi Aemili Çocuklarda Hematopoetik Kök Hücre Trasnplantasyonu Sonuçları Ve Fludarabin Içeren Hazırlık Rejiminin Transplantasyon Sonuçları Üzerine Etkisi
Abstract
FA is a disease which presents with bone marrow insufficiency, congenital malformations and predilection for cancers and which usually shows an autosomal recessive pattern of inheritance. Fanconi anemia (FA) is the most common cause of inherited bone marrow insufficiency. Hematopoietic stem cell transplantation (HSCT) is the only curative treatment method in cases who develop bone marrow insufficiency or hematologic malignancy. This study is planned to evaluate the outcomes of HSCT for FA in 34 patients to identify the effects of conditioning regimen with or without fludarabine on the outcomes. Fludarabine containing conditioning regimen was given in 25 patients and conditioning regimen without fludarabine was given in 9 patients. Neutrophil engraftment was seen in 97.1% of cases, thrombocyte engraftment was seen in 94.1% of cases. Graft versus host disease (GVHD) was developed in 21.2% of all cases; this ratio was lower in patients who took fludarabine (8%) when compared with the