Hematopoietic Stem Cell Transplantation in Pediatric Myelodysplastic Syndromes
Abstract
Myelodysplastic syndromes (MDSs) are a heterogenous group of hemopoietic clonal disorders characterized by ineffective hemopoiesis and frequent evolution to leukemia.They are rare entities, particularly in children.Recently,they have been classified into 3 major groups: MDS, juvenile myelomonocytic leukemia, and Down syndromeassociated myeloid leukemia.Haematopoietic stem cell transplantation(HSCT) is the treatment of choice and results in cure rates of around 60%.
Keywords
hematopoietic stem cell transplantation, children, MDS, JMML, RAEB
Pediatrik Miyelodisplastik Sendromunda Kök Hücre Nakli
Öz
Miyelodisplastik sendromlar inefektif hematopoez,lösemi gelişme riski ile karakterize klonal heterojen bir hastalık grubudur. Çocuklarda nadiren görülür.Yakın zamanda yapılan sınıflamada 3 ana hastalık tanımlamaktadır: Jüvenil miyelomonositik lösemi (JMML),Down sendromunun miyeloid lösemisi (MDS,geçici miyeloproliferatif hastalık, ve akut miyeloid lösemi) ve MDS. Hematopoietik kök hücre nakli tek tedavi seçeneği olup, kür %60 olarak bildirilmiştir.
Anahtar Kelimeler
hematopoietik kök hücre nakli,MDS,RAEB,JMML,çocukluk çağı