title: Outcome of essential cryofibrinogenaemia in a series of 61 patients.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Internal Medicine, Rouen University Hospital, 76031 Rouen Cedex, France. cristina.belizna@wanadoo.fr
authors.forename: C C
authors.initials: CC
authors.lastname: Belizna
authors.forename: F
authors.initials: F
authors.lastname: Tron
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: M
authors.initials: M
authors.lastname: Godin
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
authors.forename: H
authors.initials: H
authors.lastname: Lévesque
mesh_terms: D000328:Adult; D000368:Aged; D003449:Cryoglobulinemia / Q000150:complications / Q000188:drug therapy* / Q000453:epidemiology; D003450:Cryoglobulins / Q000032:analysis*; D005260:Female; D015241:Fibrinogens, Abnormal / Q000032:analysis*; D005602:France / Q000453:epidemiology; D006526:Hepatitis C / Q000175:diagnosis; D006801:Humans; D007239:Infections / Q000150:complications; D008223:Lymphoma / Q000097:blood / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome
chemical_list: D003450:Cryoglobulins; D015241:Fibrinogens, Abnormal; C041874:cryofibrinogen