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骨髓增殖性综合征

[Myeloproliferative syndromes].

作者信息

Ganser A

机构信息

Medizinische Hochschule, Abteilung für Hämatologie und Onkologie, Hannover.

出版信息

Ther Umsch. 1996 Feb;53(2):97-102.

PMID:8629270
Abstract

Myelodysplastic syndromes [MDS] are clonal disorders of hematopoietic stem cells leading to a deregulation of proliferation and differentiation of the bone marrow cells. Clinically the patients present with symptoms and signs of anemia, thrombocytopenia, and neutropenia. About a third of the patients will develop acute myeloid leukemia. Supportive care is the mainstay of therapy in these mostly elderly patients. G-CSF should only be given in cases of neutropenia and infection, but not prophylactically. Selected patients with severe or transfusion-dependent anemia will respond to treatment with erythropoietin. In advanced MDS aggressive chemotherapy should be considered, while in patients below 50 years of age and an HLA-identical sibling donor allogeneic bone marrow transplantation is the treatment of choice.

摘要

骨髓增生异常综合征(MDS)是造血干细胞的克隆性疾病,可导致骨髓细胞增殖和分化失调。临床上,患者表现出贫血、血小板减少和中性粒细胞减少的症状和体征。约三分之一的患者会发展为急性髓系白血病。支持性治疗是这些大多为老年患者的主要治疗方法。粒细胞集落刺激因子(G-CSF)仅应在中性粒细胞减少和感染的情况下使用,而不应预防性使用。部分患有严重贫血或依赖输血的贫血患者对促红细胞生成素治疗有反应。对于晚期MDS患者,应考虑积极化疗,而对于年龄在50岁以下且有HLA匹配同胞供体的患者,异基因骨髓移植是首选治疗方法。

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