Chronic Intravascular Coagulation Syndrome

TLDR

The syndrome is a chronic intravascular coagulation disorder marked by fibrinogen and platelet depletion with no evidence of other clotting factors or secondary fibrinolysis, and the presence of a cold‑precipitable cryoprecipitate helped establish the diagnosis and gauge coagulation intensity. Heparin controlled the syndrome, whereas warfarin failed to prevent thrombotic events; a persistent cryofibrinogen and a β1 globulin (~50 % of total protein) that forms a reversible cold‑precipitable complex with fibrinogen were identified, and the cryoprecipitate’s presence reflected the severity of intravascular coagulation.

Abstract

In a patient with a chronic intravascular coagulation syndrome characterized by depletion of fibrinogen and platelets, participation of other clotting factors or secondary fibrinolysis were not demonstrable. Heparin was effective in controlling the syndrome, which was evident 10 months before discovery of a primary ovarian carcinoma. Warfarin did not prevent thrombotic complications. A plasma cryoprecipitate ("cryofibrinogen") was constantly present. Physicochemical studies demonstrated, in addition to fibrinogen, a β1 globulin amounting to approximately 50 per cent of the total protein. The β1 globulin, presumably an "incomplete cryoglobulin," formed a reversible, cold-precipitable complex with the fibrinogen and was immunochemically related to a normal serum protein. The reason for the association of the cryoprecipitate with the intravascular coagulation process was not determined, but its presence helped to establish the diagnosis and reflected the intensity of intravascular coagulation during heparin and other therapy.