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反应性噬血细胞综合征对联合化疗及类固醇冲击疗法有效

[Reactive hemophagocytic syndrome responded to combination chemotherapy with steroid pulse therapy].

作者信息

Okuda T, Yumoto Y

机构信息

Division of Hematology and Cancer Chemotherapy, Nagahama City Hospital, Japan.

出版信息

Rinsho Ketsueki. 1995 Nov;36(11):1316-20.

PMID:8691575
Abstract

A 66-year-old male was admitted to our hospital because of persistent fever. Laboratory data revealed leukopenia, thrombocytopenia, marked elevation of serum lactic acid dehydrogenase and ferritin levels, as well as disseminated intravascular coagulophathy (DIC). Bone marrow aspiration showed increased numbers of mature histiocytes with hemophagocytosis and a diagnosis of reactive hemophagocytic syndrome was made. In the broad spectrum of this syndrome, we suspected virus-associated hemophagocytic syndrome (VAHS) but no causative viral infection was detected. Since DIC is known to be a poor prognostic factor, he was given combination chemotherapy containing VP-16 in addition to pulse therapy of methylprednisolone. He completely recovered after the treatment. Chemotherapy is one option in the treatment of adult onset reactive hemophagocytic syndrome.

摘要

一名66岁男性因持续发热入住我院。实验室检查数据显示白细胞减少、血小板减少、血清乳酸脱氢酶和铁蛋白水平显著升高,以及弥散性血管内凝血(DIC)。骨髓穿刺显示成熟组织细胞数量增加并伴有噬血细胞现象,诊断为反应性噬血细胞综合征。在该综合征的广泛范畴内,我们怀疑是病毒相关性噬血细胞综合征(VAHS),但未检测到致病病毒感染。由于已知DIC是一个不良预后因素,除给予甲泼尼龙冲击治疗外,还给予了包含VP - 16的联合化疗。治疗后他完全康复。化疗是成人起病的反应性噬血细胞综合征治疗的一种选择。

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