CRYOFIBRINOGENEMIA DUE TO MONOCLONAL ANTI FIBRINOGEN ANTIBODIES

History and clinical findings: For nine years a 54-year-old woman had been suffering from worsening treatment-resistant cold-dependent purpu ra of the limbs as well as cutaneous ulcerations and arthralgia, which recently had occurred even at a even slight decrease in room temperat ure. Investigations: A special form of cryofibrinogenaemia was identif ied by affinity-chromatographic separation of a plasma cryoprecipitate . From this cryoprecipitate a monoclonal antifibrinogen antibody (IgG- kappa.) was isolated which, in the cold, formed a precipitating comple x with fibrinogen. Paraproteinaemia was not demonstrated by convention al serum and plasma electrophoresis. There was no evidence of neoplasm a. Treatment and course: Attempted treatment with steroids, fibrinolyt ic agents and intravenous cyclophosphamide was unsuccessful. But long- term repeated plasmaphereses and antiimmunoglobulin adsorption improve d the symptoms. After 5 years of this treatment - 14 years after onset of symptoms - the patient died of the consequences of fulminant pulmo nary embolism. Conclusion: To establish the diagnosis of monoclonal cr yofibrinogenaemia it is necessary, first, to identify the cryoprecipit ate in plasma: secondly, to undertake affinity-chromatographic separat ion of the cryoprecipitate with subsequent analysis of its components.