摘要
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 Down syndrome （trisomy 21） and occurs at an incidence of up to 10%. Typical for this entity is a proliferation of myeloid blasts with megakaryoblastic and/ or erythroblastic features detectable in the peripheral blood. In the majority of cases rIMS is asymptomatic, and only a small percentage of children show clinical symptoms due to hyperleukocytosis, thrombocytopenia, anemia, or hepatomegaly. Almost all children will achieve spontaneous remission after 2-4 months （disappearance of myeloid blasts and regression of clinical symptoms） . Patients with initial hyperleukocytosis and hepatomegaly, however, carry a particular risk of developing hepatopathy followed frequently by lethal liver fibrosis. Chemotherapy with cytarabine is indicated in patients with clinical symptoms of hyperleuk0cytosis or early signs of hepatopathy. Within the first 4 years the risk of developing acute myeloblastic leukemia （AML） is high at 20- 30%; therefore, regular followups are advised. This analysis is based on one of the largest databases for TMS.
