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[获得性血管性血友病综合征与恶性淋巴瘤相关]

[Acquired von Willebrand's syndrome associated with malignant lymphoma].

作者信息

Schneider P, Tran-Thang C, Maillard M, Bachmann F

出版信息

Schweiz Med Wochenschr. 1979 Sep 29;109(37):1390.

PMID:314665
Abstract

The sudden development of multiple bleeding episodes in a 63-year-old patient with malignant lymphoma was shown to be due to the presence of an acquired von Willebrand syndrome, characterized by a prolonged bleeding time and low levels of the factors VIII: C, VIIR:Ag and VIII:Rcof. Crossed immunoelectrophoresis demonstrated diminution of the factor VIII portion with least anodic mobility. Mixing experiments of the patient's and normal plasma revealed a weak (T/2: 4.5 hours) inhibitory activity against the factors VIII:C, VIIIR:Ag and VIII:Rcof. After infusion of 800 units of cryoprecipitate there was rapid destruction (T/2: less than 1 hour) of infused factors VIII: C and VIIIR:Ag and no correction of the bleeding time. Chemotherapy resulted in disappearance of the lymphoma and the hemostatic defect, and its discontinuation in their reappearance. The weak inhibitory activity of the patient's plasma does not fully account for the factor VIII levels observed and other pathophysiological mechanisms, such as adsorption of factor VIII to the malignant lymphocytes, must be considered.

摘要

一名63岁恶性淋巴瘤患者突然出现多次出血发作,结果显示这是由获得性血管性血友病综合征所致,其特征为出血时间延长以及VIII:C、VIIR:Ag和VIII:Rcof因子水平降低。交叉免疫电泳显示,阳极迁移率最低的VIII因子部分减少。患者血浆与正常血浆的混合实验显示,对VIII:C、VIIIR:Ag和VIII:Rcof因子具有微弱的(T/2:4.5小时)抑制活性。输注800单位冷沉淀后,输注的VIII:C和VIIIR:Ag因子迅速被破坏(T/2:少于1小时),出血时间未得到纠正。化疗导致淋巴瘤和止血缺陷消失,化疗停止后它们又再次出现。患者血浆的微弱抑制活性并不能完全解释所观察到的VIII因子水平,必须考虑其他病理生理机制,如VIII因子吸附到恶性淋巴细胞上。

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