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首页> 外文期刊>Journal of clinical rheumatology >Cryofibrinogenemia triggered by a monoclonal paraprotein successfully treated with cyclophosphamide
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Cryofibrinogenemia triggered by a monoclonal paraprotein successfully treated with cyclophosphamide

机译：由环磷酰胺成功治疗的单克隆副蛋白引发的冷冻原纤维蛋白血症

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摘要

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.
机译：冷冻原纤维蛋白原血症是一种罕见的临床发现，其生理致病机理尚不清楚。我们描述了一名女性，该患者患有感冒引起的广泛坏死性病变，对最初的皮质类固醇和抗凝治疗反应较差。血清冰球蛋白测定持续阴性。经过数年的进化，她出现了严重的与寒冷有关的皮肤病变，需要截肢。进一步的分析揭示了冷冻原蛋白原的存在和明显无意义的血清单克隆免疫球蛋白Gκ峰。我们还证明了纤维蛋白原的冷沉淀与单克隆副蛋白的存在直接相关。病变对静脉注射环磷酰胺和地塞米松的B细胞靶向治疗反应显着。

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