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一例以骨髓增殖性综合征告终的阵发性夜间血红蛋白尿病例。

A case of paroxysmal nocturnal haemoglobinuria terminating in a myeloproliferative syndrome.

作者信息

Boyd A W, Parkin J D, Castaldi P A

出版信息

Aust N Z J Med. 1979 Apr;9(2):181-3. doi: 10.1111/j.1445-5994.1979.tb04325.x.

Abstract

This report discusses the case of a 60-year-old man who presented in 1969 with thrombocytopenia and mild marrow hypoplasia. A diagnosis of paroxysmal nocturnal haemoglobinuria (PNH), was established. The subsequent course included episodes of overt intravascular haemolysis. Thrombocytopenia reverted on several occasions during Oxymetholone therapy. The terminal phase of the illness was marked by the development of a leukocytosis and densely hypercellular bone marrow with splenomegaly. The features were those of a myeloproliferative disorder, although frank leukaemia did not develop.

摘要

本报告讨论了一名60岁男性的病例,该患者于1969年出现血小板减少和轻度骨髓发育不全。确诊为阵发性夜间血红蛋白尿(PNH)。随后的病程包括明显的血管内溶血发作。在使用羟甲烯龙治疗期间,血小板减少症曾多次恢复。疾病的终末期表现为白细胞增多、骨髓细胞极度增生伴脾肿大。这些特征符合骨髓增殖性疾病,尽管未发展为明显的白血病。

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