Erschienen in:
01.09.2015 | Review Article
Myeloid proliferations associated with Down syndrome
verfasst von:
Alan B. Cantor
Erschienen in:
Journal of Hematopathology
|
Ausgabe 3/2015
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Abstract
A subset of Down syndrome (DS) (trisomy 21) neonates is born with a unique erythromegakaryocytic myeloproliferative disorder that spontaneously resolves over the first few months of life (DS-transient abnormal myelopoiesis (DS-TAM); previously called DS-transient myeloproliferative disorder (DS-TMD) and DS-transient leukemia (DS-TL)). These infants are at high risk for developing subsequent acute megakaryoblastic leukemia (myeloid leukemia associated with Down syndrome (ML-DS); previously called DS-acute megakaryoblastic leukemia (DS-AMKL)). The molecular basis for DS-TAM/ML-DS remained mysterious for a long period of time. However, new genetic insights have been gained over the past 12 years that have begun to decipher the pathophysiology of this unusual disorder.