Down Sendromunun Geçici Miyeloproliferatif Hastalığı: Vaka Sunumu

Down sendromlu çocuklar 10 ila 20 kat artmış akut lösemi gelişme riskine sahiptir. Down sendromunun geçici miye- loproliferatif hastalığı DS-GMPH , Down sendromlu DS yenidoğanların % 10-20’sinde görülür. DS-GMPH’lu vakaların yaklaşık % 30’unda ilk üç yılda akut megakar- yoblastik lösemi AML-M7 gelişir. DS-GMPH’nın akut lösemiden ayrıcı bulgusu, kemik iliğindeki blast sayısının periferik kandakinden daha az olmasıdır. Bu vaka rapo- runda; DS-GMPH tanısı almış bir yenidoğan sunulmuştur. Down sendromlu yenidoğanlarda, özellikle de lökositozu veya trombositopenisi bulunanların periferik kan yayması incelemesinin, akut lösemi veya DS-GMPH yönünden önemi vurgulanmak istendi

Anahtar Kelimeler:

Down sendromu, geçici miyeloproliferatif hastalık, akut megakaryoblastik lösemi

Case report: Transient Myeloproliferative Disorder of Down Syndrome

Children with Down syndrome have a 10- to 20-fold incre- ased risk of developing leukemia. Transient myeloprolife- rative disorder of Down syndrome TMD-DS occurs in 10-20 % of Down syndrome DS newborns. Acute mega- karyoblastic leukemia AML-M7 develops in approxima- tely 30 % of the cases with TMD-DS within the first three years of life. The distinctive finding of TMD-DS from acute leukemia is decreased percentage of the blasts in bone marrow compared to peripheral blood. In this case report, a newborn with TMD-DS is presented. We emphasize the importance of the peripheral blood smear examination in newborns with DS, especially with leucocytosis or throm- bocytopenia in order to diagnose TMD-DS or acute leuke- mia

Keywords:

Down syndrome, transient myeloproliferative disorder, acute megakaryoblastic leukemia,

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