/transient myeloproliferative syndrome

transient myeloproliferative syndrome

Rare Disease

MONDO:0008040

Also Known As

MSTTAMTMDTransient abnormal myelopoiesis associated with Down syndromeleukemia, transient, of Down syndrometransient abnormal myelopoiesistransient leukaemiatransient leukemiatransient leurkemia of Down syndrometransient myeloproliferative diseasetransient myeloproliferative disordertransient myeloproliferative syndrometransient myeloproliferative syndrome (disease)Mstleukemia, transientmyeloproliferative syndrome, transient

Definition

A myeloid proliferation occurring in newborns with Down syndrome. It is clinically and morphologically indistinguishable from acute myeloid leukemia and is associated with GATA1 mutations. The blasts display morphologic and immunophenotypic features of megakaryocytic lineage. In the majority of patients the myeloid proliferation undergoes spontaneous remission.