Cryofibrinogenemia: an addition to the differential diagnosis of calciphylaxis in end-stage renal disease

Abstract

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.