Çocuklarda Miyelodisplastik Sendrom

Abstract

Miyelodisplastik sendrom (MDS); sitopeni, displazi ve inefektif eritropoyez ile karakterize klonal bir kök hücre bozukluğudur. Çocuklarda MDS erişkinlere göre oldukça nadir görülür. Miyelodisplastik sendromda en sık sitopeni ile ilişkili şikayetler görülür. Kanama, enfeksiyon ve solukluk sık bulgulardır. Tanı klinik bulgular, morfoloji, immünfenotip ve sitogenetik tetkiklerle konulur. Açıklanamayan uzun süren sitopeni, en az bir seride displazi, klonal sitogenetik anomali ve artmış blast sayısı tanı kriterleridir ve en az ikisinin olması tanıda yol göstericidir. Çocukluk çağı ve erişkin MDS arasında hem sınıflama, hem altta yatan hastalıklar hem de tedavi yaklaşımları açısından önemli farklılıklar vardır. Bu yazıda erişkin dönemde ortaya çıkan MDS ile karşılaştırılarak çocukluk çağında MDS anlatılmıştır.

Keywords

• Miyelodisplastik sendrom
• Çocuk
• Displazi
• Sitopeni

Myelodysplastic Syndrome in Children

Abstract

Myelodysplastic syndrome (MDS); is a clonal stem cell disorder characterized by cytopenia, dysplasia and ineffective erythropoiesis. MDS is very rare in children compared to adults. In myelodysplastic syndrome, complaints related to cytopenia such as bleeding, infection, and pallor are the most common. Diagnosis is made through clinical findings, morphology, immunophenotype and cytogenetic methods. Unexplained prolonged cytopenia, dysplasia in at least one series, clonal cytogenetic anomaly and increased blasts are diagnostic criteria, and the presence of at least two guides diagnosis. There are important differences between childhood and adult MDS in terms of classification, underlying diseases and treatment approaches. In this article, MDS in childhood is described by comparing it with adult MDS.

Keywords

• Myelodysplastic syndrome
• Child
• Dysplasia
• Cytopenia

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