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Rheumatology 2008 47(2):205-207; doi:10.1093/rheumatology/kem341
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© The Author 2008. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oxfordjournals.org

Outcome of essential cryofibrinogenaemia in a series of 61 patients

C. C. Belizna1,2, F. Tron3, P. Joly4, M. Godin5, M. Hamidou6 and H. Lévesque1

1Department of Internal Medicine, Rouen University Hospital, 2INSERM U644, Rouen Faculty of Medicine and Pharmacy, 3Immunology Department, 4Dermatology Department, 5Nephrology Department, Rouen University Hospital, Rouen, France and 6Department of Internal Medicine, Nantes University Hospital, Nantes, France.

Correspondence to: Dr C. Belizna, Department of Internal Medicine, Rouen University Hospital, 76031 Rouen Cedex, France. E-mail: cristina.belizna{at}wanadoo.fr


  Abstract

Objectives. The present study assessed the outcome of several cases of cryofibrinogenaemia detected in our hospitals during a 10-yr period (December 1996–April 2007), and also attempted to evaluate the clinical manifestations and associated diseases.

Methods. We performed a retrospective study in a series of 61 consecutive cryofibrinogenemia patients detected in our hospitals.

Results. In the 61 cryofibrinogenaemia patients, 18 had essential cryofibrinogenaemia and 43 secondary cryofibrinogaemia. Five out of the 18 patients with primary cryofibrinogaemia (27%) developed lymphoma after a 5-yr follow-up period. The main manifestations were cutaneous, and there were no differences in clinical presentation and disease severity in both types of cryofibrinogenaemia. A small number of patients (six) had cryofibrinogenaemia associated with cryoglobulinaemia, and in two cases, hepatitis C virus infection was detected; but no differences were observed between these two groups of patients.

Conclusion. Cryofibrinogenaemia was found in our study with a high prevalence, suggesting that this pathology is rather underestimated.

Our data further suggests that these patients should have a regular follow-up because of the high risk of symptom recurrence. We also hypothesize that in some cases essential cryofibrinogenaemia might be a prerequisite for a secondary disease.

KEY WORDS: Cryofibrinogenaemia, Essential cryofibrinogenaemia, Outcome

Submitted 28 February 2007; revised version accepted 19 November 2007.
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