Sankarasubbaiyan S, Scott G, Holley J L
Nephrology Unit, University of Rochester Medical Center, NY 14642, USA.
Am J Kidney Dis. 1998 Sep;32(3):494-8. doi: 10.1053/ajkd.1998.v32.pm9740168.
Cryofibrinogenemia is a disorder characterized by cryoprecipitation with variable clinical presentation that was first described by Korst and Kratochvil in 1955. Cryofibrinogen is a cold insoluble complex of fibrin, fibrinogen, and fibrin split products with albumin, cold insoluble globulin, factor VIII, and plasma proteins. Cryofibrinogenemia is associated with metastatic malignancies, collagen vascular diseases, and thromboembolic disorders and may be clinically asymptomatic or present with thromboembolic phenomena of skin and viscera. The pathogenesis of cryofibrinogenemia is unknown. It may be caused by the inhibition of fibrinolysis, leading to an accumulation of cryofibrinogen. Treatment of cryofibrinogenemia may include Stanozolol, plasmapheresis, and fibrinolytics. Cryofibrinogenemia simulates calciphylaxis clinicopathologically, because both may present with skin necrosis. Calciphylaxis has been reported in end-stage renal disease, but we report the first case of cryofibrinogen in a chronic dialysis patient. We suggest that in the appropriate clinical setting, cryofibrinogenemia should be considered in the differential diagnosis of calciphylaxis, and serum cryofibrinogen levels should be measured in end-stage renal disease patients presenting with skin necrosis.
冷纤维蛋白原血症是一种以冷沉淀为特征且临床表现多样的病症，于1955年由科斯特和克拉托赫维尔首次描述。冷纤维蛋白原是一种由纤维蛋白、纤维蛋白原、纤维蛋白裂解产物与白蛋白、冷不溶性球蛋白、凝血因子VIII和血浆蛋白组成的冷不溶性复合物。冷纤维蛋白原血症与转移性恶性肿瘤、胶原血管疾病和血栓栓塞性疾病相关，临床症状可能不明显，也可能表现为皮肤和内脏的血栓栓塞现象。冷纤维蛋白原血症的发病机制尚不清楚。它可能是由纤维蛋白溶解受抑制导致冷纤维蛋白原蓄积引起的。冷纤维蛋白原血症的治疗方法可能包括司坦唑醇、血浆置换和纤溶剂。冷纤维蛋白原血症在临床病理上类似于钙过敏，因为两者都可能出现皮肤坏死。钙过敏在终末期肾病中已有报道，但我们报告了首例慢性透析患者出现冷纤维蛋白原血症的病例。我们建议，在适当的临床情况下，冷纤维蛋白原血症应纳入钙过敏的鉴别诊断中，对于出现皮肤坏死的终末期肾病患者，应检测其血清冷纤维蛋白原水平。
