Objective: To explore the clinical characteristics and outcome of patients with type Ⅱ cryoglobulinemia in our center. Methods: Clinical data of 61 patients diagnosed with type Ⅱ cryoglobulinemia in Peking Union Medical College hospital from May 2015 to January 2020 were retrospectively analyzed. Results: A total of 61 patients were enrolled in the study, including 26 (42.6%) males, with a median age of 53 (range 28-79) years. The primary diseases identified were hepatitis C virus infection (21.3%) , hepatitis B virus infection (21.3%) , autoimmune diseases (14.8%) , and hematological tumors (11.5%) . Idiopathic cryoglobulinemia patients accounted for 19 cases (31.1%) . The major symptoms presented were purpura, proteinuria, hematuria, renal failure, fever and arthralgia. The median concentration of cryoglobulin level was 215.9 (22.0-17 075.8) g/L, and the IgM-monoclonal component of cryoglobulin was identified in 54 patients (88.5%) . Rheumatoid factor increased in 93.2% of patients. C3 decreased in 57.6% of patients. C4 decreased in 61.0% of patients. Treatment was initiated in 49 patients (80.3%) , and the total clinical remission rate was 75.5%. The expected 3-year overall survival was 89.3%. Conclusion: Type Ⅱ cryoglobulinemia was a systemic disease with multi-organ involvement. Most type Ⅱ CGs were secondary to hepatitis virus infection. Early diagnosis and proper treatment could bring better outcome.
目的： 探讨Ⅱ型冷球蛋白血症患者的临床特征及预后。 方法： 回顾性分析2015年5月至2020年1月北京协和医院确诊的61例Ⅱ型冷球蛋白血症患者的临床资料。 结果： 61例患者中，男性26例（42.6%），中位诊断年龄为53（28~79）岁。继发病因包括丙型肝炎病毒（HCV）感染（21.3%）、乙型肝炎病毒（HBV）感染（21.3%）、自身免疫性疾病（14.8%）和血液系统肿瘤（11.5%）。31.1%患者为特发性。常见首诊症状包括皮肤紫癜、蛋白尿、血尿、肾功能不全、发热及关节痛。实验室检查显示，中位冷球蛋白水平为215.9（22.0~17 075.8）g/L，54例（88.5%）为IgM单克隆。类风湿因子（RF）升高患者占93.2%，C3下降患者占57.6%，C4下降患者占61.0%。共49例（80.3%）患者接受治疗，总体临床缓解率为75.5%，预计3年总生存率为89.3%。 结论： Ⅱ型冷球蛋白血症是一种多系统受累的全身性疾病，病因以肝炎病毒感染多见。早期诊断和干预对于改善预后有重要意义。.
Keywords: Cryoglobulin; Hepatitis virus; Type Ⅱ cryoglobulinemia.