Zeuner R A, Béress R, Schröder J O, Gutschmidt H J, Christophers E, Euler H H
II. Medizinische Klinik und Poliklinik der Universität, Kiel.
Dtsch Med Wochenschr. 1996 Sep 6;121(36):1084-9. doi: 10.1055/s-2008-1043110.
For nine years a 54-year-old woman had been suffering from worsening treatment-resistant cold-dependent purpura of the limbs as well as cutaneous ulcerations and arthralgia, which recently had occurred even at a even slight decrease in room temperature.
A special form of cryofibrinogenemia was identified by affinity-chromatographic separation of a plasma cryoprecipitate. From this cryoprecipitate a monoclonal antifibrinogen antibody (IgG-kappa) was isolated which, in the cold, formed a precipitating complex with fibrinogen. Paraproteinaemia was not demonstrated by conventional serum and plasma electrophoresis. There was no evidence of neoplasma.
Attempted treatment with steroids, fibrinolytic agents and intravenous cyclophosphamide was unsuccessful. But long-term repeated plasmaphereses and anti-immunoglobulin adsorption improved the symptoms. After 5 years of this treatment-14 years after onset of symptoms-the patient died of the consequences of fulminant pulmonary embolism.
To establish the diagnosis of monoclonal cryofibrinogenemia it is necessary, first, to identify the cryoprecipitate in plasma; secondly, to undertake affinity-chromatographic separation of the cryoprecipitate with subsequent analysis of its components.
一名54岁女性九年来一直患有治疗抵抗性且病情逐渐加重的肢端冷依赖性紫癜，伴有皮肤溃疡和关节痛，近期甚至在室温稍有下降时就会发作。
通过血浆冷沉淀物的亲和色谱分离法，确定了一种特殊形式的冷纤维蛋白原血症。从该冷沉淀物中分离出一种单克隆抗纤维蛋白原抗体（IgG-κ），该抗体在低温下与纤维蛋白原形成沉淀复合物。常规血清和血浆电泳未显示副蛋白血症。未发现肿瘤迹象。
使用类固醇、纤溶药物和静脉注射环磷酰胺进行治疗均未成功。但长期反复进行血浆置换和抗免疫球蛋白吸附改善了症状。经过5年这种治疗——症状出现14年后——患者死于暴发性肺栓塞的后果。
要确诊单克隆冷纤维蛋白原血症，首先需要识别血浆中的冷沉淀物；其次，要对冷沉淀物进行亲和色谱分离并随后分析其成分。
