32例冷球蛋白血症患者临床、血清学和病理学特点

Clinical, Laboratory and Pathological Features of 32 Cases of Cryoglobulinemia

为提高对冷球蛋白血症(cryoglobulinemia, CG)的识别率和诊治率,通过回顾性分析方法,研究了2011年11月至2016年6月四川大学华西医院确诊的32例CG患者的临床、血清学及病理学资料。结果表明:32例CG患者中,自身免疫相关22例,感染相关14例,肿瘤相关5例,原发性1例。CG最常累及皮肤(25例,占78%),其次为甲状腺受累21例(66%)、肾脏受累20例(63%)、疲乏19例(59%)等。肿瘤、感染相关CG均以补体C3降低显著,自身免疫相关CG以补体C4降低多见。肾脏受累、肢端发绀均与补体降低呈负相关。CG皮肤受累的病理特征为小血管白细胞破碎性血管炎和(或)淋巴细胞性血管炎,肾脏受累可表现为各种病理类型的肾小球肾炎。肿瘤相关CG预后最差。可见CG涉及疾病谱广泛,累及器官系统多样,以皮肤受累最常见,CG的血清免疫学指标中,补体降低、γ球蛋白升高、类风湿因子(rheumatoid factor, RF)升高最常见,CG皮肤及肾脏受累的病理表现异质性高,早识别、早诊治有助于改善CG预后。

The research aims to improve the diagnosis and treatment of cryoglobulinemia(CG). Through retrospective analysis, all clinical, serological and pathological data of 32 patients with positive cryoglobulin diagnosed in West China Hospital of Sichuan University from November 2011 to June 2016 were studied. The Results show that among 32 patients, 22 are autoimmune disorders, 14 are infection, 5 are malignancy, and 1 is primary. The most frequently involved organ of CG is skin, with 25 cases(about 78%), followed by thyroid in 21 cases(66%), kidney in 20 cases(63%), and fatigue in 19 cases(59%). Low serum complement C3 is more common in both malignancy and infection related CG, while low serum complements C4 is more common in autoimmune disease related CG. Kidney injury and acral cyanosis are negatively correlated with low serum complement. The pathological characteristics of cutaneous biopsy are leukocytoclastic vasculitis and/or lymphocytic vasculitis. Renal pathological characteristics are diverse types of glomerulonephritis. Tumor related CG has the worst prognosis. It is concluded that the etiological spectrum of CG is extensive. Multi-organs/systems, particularly the skin, are involved. Low complement levels, high levels of serum γ globulin and rheumatoid factor(RF) are commonly observed in CG. The pathological manifestations of skin and kidney involvement are highly heterogeneous. Early diagnosis and treatment are helpful to improve the prognosis.