Hematopoietic Stem Cell Transplantation in Pediatric Myelodysplastic Syndromes

Yıl 2014, , 82 - 89, 30.10.2014

Gülay Sezgin

https://doi.org/10.17827/aktd.41632

Öz

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 syndrome–associated myeloid leukemia.Haematopoietic stem cell transplantation(HSCT) is the treatment of choice and results in cure rates of around 60%.

Anahtar Kelimeler

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

Kaynakça

• Niemeyer CM, Kratz CP, Hasle H. Pediatric myelodysplastic syndromes. Curr Treat Options Oncol. 2005; 6:209-14.
• Hasle H, Niemeyer CM, Chessells JM, Baumann I, Bennett JM, Kerndrup G et al. A pediatric approach to the WHO classification of myelodysplastic and myeloproliferative diseases. Leukemia. 2003; 17:277-82.
• Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A et al. The 2008 revisionof the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009; 114:937-51.
• Mandel K, Dror Y, Poon Y Freedman MH.. A practical,comprehensive classification for pediatric myelodysplastic syndromes:the CCC system. J Pediatr Hematol Oncol. 2002; 24:596-605.
• Greenberg PL, Tuechler H, Schanz J, Sanz G, Garcia-Manero G, Solé F. Revised international prognostic scoring system for myelodysplastic syndromes. Blood. 2012; 120:2454-65.
• Niemeyer CM, Arico M, Basso G, Biondi A, Cantu Rajnoldi A, Creutzig U et al. Chronic myelomonocytic leukemia in childhood: a retrospective analysis of 110 cases. European Working Group on Myelodysplastic Syndromes in Childhood (EWOG-MDS). Blood. 1997; 89:3534-43. Niemeyer CM, Kratz CP. Pediatric myelodysplastic syndromes and and juvenile myelomonocytic leukemia:Molecular classification and treatment options. Br J Hematol. 2008; 140:610-24. Pinkel D. Differentiating juvenile myelomonocytic leukemia from infectious disease. Blood. 1998; 91:365-7.
• Locatelli F, Nöllke P, Zecca M, Korthof E, Lanino E, Peters C et al. Hematopoietic stem cell transplantation (HSCT) in children with juvenile myelomonocytic leukemia (JMML): results of the EWOG-MDS/EBMT trial. Blood. 2005; 105:410-9.
• Locatelli F, Niemeyer C, Angelucci E, Bender-Götze C, Burdach S, Ebell W et al. Allogeneic bone marrow transplantation for chronic myelomonocytic leukemia in childhood: a report from the European Working Group on Myelodysplastic Syndrome in Childhood. J Clin Oncol. 1997; 15:566