[Cryofibrinogenemia]

Rev Med Interne. 2011 May;32(5):287-91. doi: 10.1016/j.revmed.2009.12.019. Epub 2010 Jun 18.
[Article in French]

Abstract

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.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Age Factors
  • Anticoagulants / therapeutic use
  • Antifibrinolytic Agents / therapeutic use
  • Biomarkers / metabolism
  • Cryoglobulinemia* / complications
  • Cryoglobulinemia* / diagnosis
  • Cryoglobulinemia* / drug therapy
  • Cryoglobulinemia* / immunology
  • Cryoglobulinemia* / physiopathology
  • Cryoglobulins / immunology
  • Diagnosis, Differential
  • Drug Therapy, Combination
  • Glucocorticoids / therapeutic use
  • Humans
  • Immunologic Factors / immunology
  • Platelet Aggregation Inhibitors / therapeutic use
  • Risk Factors
  • Sex Factors
  • Skin / pathology
  • Treatment Outcome

Substances

  • Anticoagulants
  • Antifibrinolytic Agents
  • Biomarkers
  • Cryoglobulins
  • Glucocorticoids
  • Immunologic Factors
  • Platelet Aggregation Inhibitors

Supplementary concepts

  • Cryofibrinogenemia