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唐氏综合征的短暂性骨髓增殖性疾病(短暂性白血病)及血液学表现

Transient myeloproliferative disorder (transient leukemia) and hematologic manifestations of Down syndrome.

作者信息

Zipursky A, Brown E J, Christensen H, Doyle J

机构信息

Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.

出版信息

Clin Lab Med. 1999 Mar;19(1):157-67, vii.

Abstract

Transient Myeloproliferative Disorder (Transient Leukemia) is found in approximately 10% of newborn infants with Down Syndrome. It is characterized by the large numbers of megakaryoblasts in the peripheral blood, variable thrombocytopenia and, in a minority of cases, by a lethal course with hydrops fetalis or progressive hepatic fibrosis. Evidence is presented that this disease is truly leukemia, which, in most cases, recovers spontaneously. There is evidence that hematopoiesis is abnormal in Down Syndrome children who do not have leukemia. These abnormalities of red cells, platelets, and granulocytes are reviewed.

摘要

短暂性骨髓增殖性疾病(短暂性白血病)在约10%的唐氏综合征新生儿中被发现。其特征为外周血中存在大量巨核母细胞、血小板减少程度不一,少数情况下会出现致死性病程,伴有胎儿水肿或进行性肝纤维化。有证据表明这种疾病是真正的白血病,在大多数情况下会自发缓解。有证据显示,没有患白血病的唐氏综合征儿童存在造血异常。本文对红细胞、血小板和粒细胞的这些异常情况进行了综述。

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