Abstract：
Cryoglobulins are immunoglobulins that undergo reversible precipitation at cold temperatures. Monoclonal type-I cryoglobulinaemia is the least frequent and is associated to hematological diseases such as multiple myeloma, Waldenström\'s macroglobulinaemia, chronic lymphocytic leukaemia and lymphoma. We describe the case of a 60-year-old female patient, who suffered from burning pain in her feet for ten months before her admission. The patient presented intermittent distal cyanosis that progressed to digital ischaemia. She also reported paresthesia in her hands, difficulty in writing, and a 26-kg-weight loss. At the physical examination, it was identified livedo reticularis, palpable purpura, and painful ecchymotic lesions in her calves and feet. Moreover, peripheral pulses were palpable and symmetrical. It was observed an atrophy of the right first dorsal interosseous and both extensor digitorum brevis, as well as a distal bilateral apalesthesia and allodynia. Both Achilles reflexes were absent. Laboratory tests revealed anemia, high erythrosedimentation rate and C-reactive protein. Serum protein electrophoresis showed a monoclonal IgG-Kappa gammopathy. The results also evidenced the presence of Bence-Jones proteinuria. The bone marrow biopsy revealed less than 10% of plasma cells, and skin biopsy informed leukocytoclastic vasculitis. The patient was treated with high-dose intravenous steroids and cyclophosphamide. The treatment showed that the skin lesions had improved, pain disappeared and motor deficit stopped its progression.
摘要：
冷球蛋白是在低温下进行可逆沉淀的免疫球蛋白。I型单克隆冷球蛋白血症是最不常见的，并且与血液系统疾病如多发性骨髓瘤有关，Waldenström巨球蛋白血症，慢性淋巴细胞白血病和淋巴瘤。我们描述了一个60岁的女性患者的案例，在入院前十个月，她的脚一直灼痛。该患者出现间歇性远端紫癜，进展为数字缺血。她还报告说她手里有感觉异常，写作困难，和26公斤的减肥。在体检时,它被确认为网状livedo，明显的紫癜,小腿和脚部有疼痛的瘀斑。此外，外周脉搏明显且对称。观察到右第一背侧骨间和指短伸肌萎缩，以及远端双侧感觉减退和异常性疼痛。两个跟腱反射都不存在。实验室检查显示贫血,高红细胞沉降率和C反应蛋白。血清蛋白电泳显示单克隆IgG-κ丙种球蛋白病。结果还证明了Bence-Jones蛋白尿的存在。骨髓活检显示浆细胞不到10%,皮肤活检告知白细胞碎裂性血管炎。患者接受大剂量静脉注射类固醇和环磷酰胺治疗。治疗显示皮损有好转,疼痛消失，运动障碍停止其进展。
