[Cryofibrinogenemia].
Cryofibrinogenemia (CF) is an unrecognized disorder and is rarely symptomatic. CF represents 10 % of the whole cryoproteins. Mean age at diagnosis is between 50 and 60 years. The skin is the most frequently involved organ, in 80 % of patients at diagnosis. Skin lesions include purpura, livedo and Raynaud's phenomenon. In severe cases, skin ulcerations or necrosis and gangrene may occur. Arterial thrombosis is frequently reported (20 to 40 % of cases). Defect in the fibrinolysis process may lead to the cryofibrinogen accumulation and clotting in small and medium arteries and provide a rationale for the use of fibrinolytic agents at least in the most severe cases. In this article, we review the clinical presentation, pathogenesis and treatment of CF.
Saadoun
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UMR 7211 Inserm U959, centre de références maladies auto-immunes, service de médecine interne II, hôpital Pitié-Salpêtrière, 75013 Paris, France. david.saadoun@psl.aphp.fr
Musset
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L
Cacoub
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P
D000367:Age Factors; D000925:Anticoagulants / Q000627:therapeutic use; D000933:Antifibrinolytic Agents / Q000627:therapeutic use; D015415:Biomarkers / Q000378:metabolism; D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology / Q000503:physiopathology; D003450:Cryoglobulins / Q000276:immunology; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000276:immunology; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D012307:Risk Factors; D012737:Sex Factors; D012867:Skin / Q000473:pathology; D016896:Treatment Outcome
D000925:Anticoagulants; D000933:Antifibrinolytic Agents; D015415:Biomarkers; D003450:Cryoglobulins; D005938:Glucocorticoids; D007155:Immunologic Factors; D010975:Platelet Aggregation Inhibitors