Document Detail

Cryofibrinogenemia: new insights into clinical and pathogenic features.
MedLine Citation:
PMID:  19958891     Owner:  NLM     Status:  MEDLINE    
OBJECTIVE: Cryofibrinogenemia is an under-recognized cryoprotein that can be life-threatening when untreated. Our aim was to describe the prevalence and clinical findings of patients with cryofibrinogenemia and to clarify the mechanisms involved. METHODS: Between 1996 and 2006, 2312 patients were tested for cryofibrinogenemia in a single university hospital. A total of 515 patients had positive test results, of whom 455 (88.3%) had an associated cryoglobulin. RESULTS: Sixty patients (11.7%) with persistent cryofibrinogenemia and without cryoglobulin were included in the study. Main clinical manifestations related to cryofibrinogenemia included purpura (46.6%), skin necrosis (36.6%), and arthralgia (31.6%) with cold sensitivity in 40%. Overall thrombotic events occurred in up to 40% of cases. Cryofibrinogen plasma concentration was 2 times greater in patients with thrombotic events (P=.012). Complications included gangrene (5%), septicemia (5%), and leg amputation (3.3%). Complete remission of cryofibrinogenemia was achieved in 78% of patients receiving antithrombotic agents, steroids, or immunosuppressants, whereas 41.6% of patients experienced a relapse after a median time of 9 months (range 7-42 months). After a mean follow-up of 85 months, 3 patients died of sepsis (n=2) and cardiovascular disease (n=1). Fibrinolysis status analyzed in a patient with cryofibrinogenemia showed an increase in fibrinolysis inhibitor levels, plasminogen activator inhibitor-1, alpha-2 macroglobulin, and euglobulin lysis time, which normalized after fibrinolytic therapy. CONCLUSION: Essential cryofibrinogenemia represents 12% of all the cryoproteins at Pitie-Salp??triere Hospital. Thrombotic events are frequent and could be associated with the amount of plasma cryofibrinogen. Defects in the fibrinolysis process might lead to cryofibrinogen accumulation and clotting in small and medium arteries.
David Saadoun; Ismail Elalamy; Pascale Ghillani-Dalbin; Damien Sene; Aurelien Delluc; Patrice Cacoub
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  The American journal of medicine     Volume:  122     ISSN:  1555-7162     ISO Abbreviation:  Am. J. Med.     Publication Date:  2009 Dec 
Date Detail:
Created Date:  2009-12-04     Completed Date:  2010-01-18     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0267200     Medline TA:  Am J Med     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1128-35     Citation Subset:  AIM; IM    
Pierre et Marie Curie-Paris 6 University, Department of Internal Medicine, H??pital Piti??-Salp??tri??re, Paris, France.
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MeSH Terms
Adrenal Cortex Hormones / therapeutic use
Amputation / statistics & numerical data
Arthralgia / epidemiology
Cold Temperature / adverse effects
Cryoglobulinemia / diagnosis*,  epidemiology,  etiology,  therapy*
Cryoglobulins / analysis
Fibrinolytic Agents / therapeutic use
Immunosuppressive Agents / therapeutic use
Leg / surgery
Middle Aged
Plasminogen Activator Inhibitor 1
Purpura / epidemiology
Raynaud Disease / epidemiology
Remission Induction
Sepsis / epidemiology,  mortality
Serum Globulins / metabolism
Skin / pathology
Thrombosis / epidemiology
Urticaria / epidemiology
alpha-Macroglobulins / analysis
Reg. No./Substance:
0/Adrenal Cortex Hormones; 0/Cryoglobulins; 0/Fibrinolytic Agents; 0/Immunosuppressive Agents; 0/Plasminogen Activator Inhibitor 1; 0/SERPINE1 protein, human; 0/Serum Globulins; 0/alpha-Macroglobulins

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