Cryofibrinogenemia triggered by a monoclonal paraprotein successfully treated with cyclophosphamide.
J Clin Rheumatol
; 20(1): 34-7, 2014 Jan.
Article
em En
| MEDLINE
| ID: mdl-24356479
ABSTRACT
Cryofibrinogenemia is a rare clinical finding with a yet unknown physiopathogenic mechanism. We describe the case of a woman with cold-induced extensive necrotic lesions that responded poorly to initial corticosteroid and anticoagulant therapies. Serum cryoglobulin determinations were persistently negative. After several years of evolution, she developed severe cold-related skin lesions that required left-leg amputation. Further analysis disclosed the presence of cryofibrinogen and an apparently insignificant serum monoclonal immunoglobulin Gκ peak. We additionally demonstrate that the cold precipitation of fibrinogen was directly related to the monoclonal paraprotein presence. The lesions responded dramatically to a B cell-targeted therapy with intravenous cyclophosphamide and dexamethasone.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Paraproteínas
/
Dexametasona
/
Crioglobulinemia
/
Ciclofosfamida
/
Anticorpos Monoclonais
Limite:
Female
/
Humans
/
Middle aged
Idioma:
En
Revista:
J Clin Rheumatol
Assunto da revista:
FISIOLOGIA
/
ORTOPEDIA
/
REUMATOLOGIA
Ano de publicação:
2014
Tipo de documento:
Article
País de afiliação:
Espanha