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Transient myeloproliferative syndrome(TMS)is a disorder of neonates with Dow n syndrome(trisomy 21)and occurs at an incidence of up to 10%.Typical for th is entity is a proliferation of myeloid blasts with megakaryoblastic and/or eryt hroblastic features detectable in the peripheral blood.In the majority of cases TMS is asymptomatic,and only a small percentage of children show clinical symp toms due to hyperleukocytosis,thrombocytopenia,anemia,or hepatomegaly.Almost all children will achieve spontaneous remission after 2-4 months(disappearanc e of myeloid blasts and regression of clinical symptoms).Patients with initial hyperleukocytosis and hepatomegaly,however,carry a particular risk of developi ng hepatopathy followed frequently by lethal liver fibrosis.Chemotherapy with c ytarabine is indicated in patients with clinical symptoms of hyperleukocytosis o r early signs of hepatopathy.Within the first 4 years the risk of developing ac ute myeloblastic leukemia(AML)is high at 20-30%;therefore,regular followup s are advised.This analysis is based on one of the largest databases for TMS.
Transient myeloproliferative syndrome (TMS) is a disorder of neonates with Dow n syndrome (trisomy 21) and occurs at an incidence of up to 10%. Typical for th is entity is a proliferation of myeloid blasts with megakaryoblastic and / or eryt hroblastic features detectable in the peripheral blood. the majority of cases TMS is asymptomatic, and only a small percentage of children show clinical symp toms due to hyperleukocytosis, thrombocytopenia, anemia, or hepatomegaly. Almost all children will achieve spontaneous remission after 2-4 months (disappearanc e of myeloid blasts and regression of clinical symptoms). Patients with initial hyperleukocytosis and hepatomegaly, however, carry a particular risk of developi ng hepatopathy followed frequently by lethal liver fibrosis. Chemotherapy with c ytarabine is indicated in patients with clinical symptoms of hyperleukocytosis or early signs of hepatopathy. Whith the first 4 years the risk of developing ac ute myeloblastic leukemia (AML) is high at 20-30%; ther efore, regular followup s are advised. This analysis is based on one of the largest databases for TMS.