伴有伤口愈合异常、酸溶性凝块及正常因子 XIII 的出血性疾病。

Bleeding disorder with abnormal wound healing, acid-soluble clots and normal factor XIII.

作者信息

Ragaz S, Kemp G, Furlan M, Beck E A

出版信息

Thromb Haemost. 1976 Dec 31;36(3):537-41.

Abstract

An unusual bleeding disorder clinically resembling factor XIII deficiency is presented. The only detectable coagulation abnormality was rapid clot dissolution in 1% monochloroacetic acid. This abnormality was ascribed to the sustained increase of a pepsin-like plasma protease which is activated at low pH. Asystematic search for similar phenomena revealed that massive blood transfusion may also enhance plasma-clot solubility in acid, possibly by release of a red cell protease. We conclude that the acid clot solubility test is not a specific indicator of factor XIII deficiency, but this simple assay is recommended for further studies of acid plasma protease activity. The diagnostic relevance and pathophysiologic importance of increased pepsin-like activity in plasma remain to be elucidated.

摘要

本文报告了一例临床上类似于因子 XIII 缺乏的不寻常出血性疾病。唯一可检测到的凝血异常是在 1% 一氯乙酸中凝块快速溶解。这种异常归因于一种在低 pH 下被激活的胃蛋白酶样血浆蛋白酶的持续增加。对类似现象的系统研究表明，大量输血也可能增强血浆凝块在酸性环境中的溶解度，可能是通过释放一种红细胞蛋白酶。我们得出结论，酸性凝块溶解度试验不是因子 XIII 缺乏的特异性指标，但建议采用这种简单的检测方法进一步研究酸性血浆蛋白酶活性。血浆中胃蛋白酶样活性增加的诊断相关性和病理生理重要性仍有待阐明。