Pediatrik Miyelodisplastik Sendromunda Kök Hücre Nakli

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

Hematopoietic Stem Cell Transplantation in Pediatric Myelodysplastic Syndromes

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,

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