原发性冷纤维蛋白原血症、白细胞破碎性血管炎和慢性紫癜。

Essential cryofibrinogenaemia, leukocytoclastic vasculitis and chronic purpura.

作者信息

Jantunen E, Soppi E, Neittaanmäki H, Lahtinen R

机构信息

Department of Medicine, Kuopio University Hospital, Finland.

出版信息

J Intern Med. 1993 Sep;234(3):331-3. doi: 10.1111/j.1365-2796.1993.tb00752.x.

DOI:10.1111/j.1365-2796.1993.tb00752.x

PMID:8354986

Abstract

Cryofibrinogenaemia refers to the presence of cold-precipitable proteins in plasma but not in serum. It is usually associated with malignancy, thromboembolic diseases or various inflammatory processes; rarely it may be essential. The most common clinical presentations of cryofibrinogenaemia are cold-intolerance, purpura, skin necrosis and ulcers. We describe a middle-aged woman with essential cryofibrinogenaemia, leukocytoclastic vasculitis, and chronic purpura for over 25 years with several exacerbations. In patients with otherwise unexplained purpura or skin necrosis, determination of plasma cryofibrinogen should be considered.

摘要

冷纤维蛋白原血症是指血浆中存在可被冷沉淀的蛋白质，而血清中不存在。它通常与恶性肿瘤、血栓栓塞性疾病或各种炎症过程相关；极少数情况下可能是原发性的。冷纤维蛋白原血症最常见的临床表现是不耐寒、紫癜、皮肤坏死和溃疡。我们描述了一位患有原发性冷纤维蛋白原血症、白细胞破碎性血管炎和慢性紫癜超过25年且病情多次加重的中年女性。对于患有其他原因不明的紫癜或皮肤坏死的患者，应考虑检测血浆冷纤维蛋白原。