Ⅱ型冷球蛋白血症的临床特征及预后

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.