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.
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 cryofibrinogenemiapatients 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.
Authors: C M Hedrich; B Fiebig; F H Hauck; S Sallmann; G Hahn; C Pfeiffer; G Heubner; Min Ae Lee-Kirsch; M Gahr Journal: Clin Rheumatol Date: 2008-06-10 Impact factor: 2.980
Authors: C Paradis; M Cadieux-Dion; C Meloche; M Gravel; J Paradis; A Des Roches; G Leclerc; P Cossette; P Begin Journal: J Clin Immunol Date: 2019-01-26 Impact factor: 8.317