Han H X, Cao X X, Su W, Shen K N, Zhang L, Zhou D B, Li J
Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Department of Clinical Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.
Zhonghua Xue Ye Xue Za Zhi. 2021 Jun 14;42(6):447-451. doi: 10.3760/cma.j.issn.0253-2727.2021.06.002.
To explore the clinical characteristics and outcome of patients with type Ⅱ cryoglobulinemia in our center. 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. 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%. 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.
探索我院Ⅱ型冷球蛋白血症患者的临床特征及预后。回顾性分析2015年5月至2020年1月在北京协和医院确诊为Ⅱ型冷球蛋白血症的61例患者的临床资料。本研究共纳入61例患者，其中男性26例（42.6%），中位年龄53岁（范围28 - 79岁）。确定的主要疾病为丙型肝炎病毒感染（21.3%）、乙型肝炎病毒感染（21.3%）、自身免疫性疾病（14.8%）和血液系统肿瘤（11.5%）。特发性冷球蛋白血症患者占19例（31.1%）。主要症状为紫癜、蛋白尿、血尿、肾衰竭、发热和关节痛。冷球蛋白水平的中位浓度为215.9（22.0 - 17075.8）g/L，54例患者（88.5%）检测到冷球蛋白的IgM单克隆成分。93.2%的患者类风湿因子升高。57.6%的患者C3降低。61.0%的患者C4降低。49例患者（80.3%）开始治疗，总临床缓解率为75.5%。预计3年总生存率为89.3%。Ⅱ型冷球蛋白血症是一种多器官受累的全身性疾病。大多数Ⅱ型冷球蛋白血症继发于肝炎病毒感染。早期诊断和恰当治疗可带来更好的预后。
