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一名左心房黏液瘤患者的波动性症状性冷纤维蛋白原血症

Fluctuating symptomatic cryofibrinogenaemia in a patient with left atrial myxoma.

作者信息

Cobcroft R G, Shearer A, Gill D S, Forgan-Smith J

机构信息

Department of Haematology, Princess Alexandra Hospital, Brisbane.

出版信息

Br J Haematol. 1994 Sep;88(1):213-4. doi: 10.1111/j.1365-2141.1994.tb05003.x.

Abstract

We report a case of left atrial myxoma associated with a fluctuating level of cryofibrinogen. Her initial symptoms and signs were consistent with primary cryofibrinogenaemia, but a repeat episode occurred without the cryofibrinogen being detectable. A more typically embolic cerebro-vascular accident (CVA) occurred, with the subsequent discovery of the patient's myxoma. The cryofibrinogen was again present but it disappeared with resection of the myxoma. We propose that the degree of activation of the coagulation system around the myxoma fluctuated, causing variable conversion of fibrinogen to fibrin, so that at times partially cross-linked fibrin(ogen) resulted in cryofibrinogen, but at other times frank emboli from more complete clotting occurred.

摘要

我们报告一例左心房黏液瘤伴冷纤维蛋白原水平波动的病例。患者最初的症状和体征与原发性冷纤维蛋白原血症相符,但在未检测到冷纤维蛋白原的情况下再次发作。随后发生了更典型的栓塞性脑血管意外(CVA),并发现了患者的黏液瘤。冷纤维蛋白原再次出现,但在黏液瘤切除后消失。我们认为,黏液瘤周围凝血系统的激活程度波动,导致纤维蛋白原向纤维蛋白的可变转化,使得有时部分交联的纤维蛋白(原)导致冷纤维蛋白原形成,但在其他时候,更完全凝血形成的明显栓子出现。

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