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卡萨巴赫-梅里特综合征的血管肉瘤变体

Angiosarcoma variant of Kasabach-Merritt syndrome.

作者信息

Alliot C, Tribout B, Barrios M, Gontier M F

机构信息

Department of Haematology, University Hospital of Amiens, France.

出版信息

Eur J Gastroenterol Hepatol. 2001 Jun;13(6):731-4. doi: 10.1097/00042737-200106000-00020.

DOI:10.1097/00042737-200106000-00020
PMID:11434603
Abstract

Kasabach-Merritt syndrome is characterized by the occurrence of disseminated intravascular coagulation (DIC) usually caused by benign angiomatous tumours. Here we report the case of a 70-year-old man in whom DIC revealed a locally advanced hepatic tumour. Although DIC resolved with heparin, antithrombin III, fresh frozen plasma and corticosteroids, the patient died from haemoperitoneum following a fall, 3 months after the initial observation. Histopathological examination by autopsy allowed the diagnosis of hepatic angiosarcoma. The physiopathogenic mechanisms and treatment options are discussed.

摘要

卡萨巴赫-梅里特综合征的特征是通常由良性血管瘤性肿瘤引起的弥散性血管内凝血(DIC)。本文报告了一例70岁男性患者,其弥散性血管内凝血提示存在局部进展性肝肿瘤。尽管使用肝素、抗凝血酶III、新鲜冰冻血浆和皮质类固醇后弥散性血管内凝血得到缓解,但患者在初次观察3个月后因跌倒导致腹腔积血死亡。尸检组织病理学检查确诊为肝血管肉瘤。文中讨论了其发病机制和治疗选择。

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