16例冷球蛋白血症患者临床病理特点分析  

Analysis of clinicopathological features in 16 patients with cryoglobulinemia

作　　者：高琛妮 沈平雁[1] 潘晓霞[1] 徐耀文[1] 徐静[1] 胡晓帆[1] 张文[1] 谢静远[1] 任红[1] 陈晓农[1] GAO Chenni;SHEN Pingyan;PAN Xiaoxia;XU Yaowen;XU Jing;HU Xiaofan;ZHANG Wen;XIE Jingyuan;REN Hong;CHEN Xiaonong(Department of Nephrology,Ruijin Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院肾脏内科

出　　处：《内科理论与实践》2019年第5期298-302,共5页Journal of Internal Medicine Concepts & Practice

摘　　要：目的:分析冷球蛋白血症的临床及病理特征,使临床医师对其有更好的认识。方法:回顾性分析2016年4月至2018年12月我院就诊,检测血清冷球蛋白结果为阳性的患者共16例。收集患者的一般情况、实验室检查结果、肾脏病理结果以及随访情况,并进行分析。结果:16例患者确诊时平均年龄(54.4±17.2)岁,男性9例(56.3%)。其中感染肝炎病毒患者4例,均为乙型肝炎病毒。淋巴浆细胞异常增殖患者8例,自身免疫疾病患者4例。患者起病表现多样,临床表现差异较大。14例患者有肾脏累及,10例患者伴肾功能受损。12例患者接受肾穿刺活组织检查(活检),其中7例患者为膜增生性肾小球肾炎(membrano-proliferative glomerulonephritis,MPGN),其他肾脏病理表现包括肾小管间质病2例,新月体肾炎2例,血栓性微血管病肾损害1例。12例患者使用肾上腺糖皮质激素伴或不伴免疫抑制剂,3例血液系统恶性肿瘤患者针对原发病进行化学治疗;7例患者联合血浆置换治疗。中位随访时间17.5个月,3例患者死亡,2例患者进入维持性透析治疗,余患者肾功能稳定。结论:冷球蛋白血症以中老年患者多见,肾脏受累普遍。由于该病进展快,预后较差,如临床有疑似本病患者应进行标准化冷球蛋白测定,尽早确诊。Objective To analyze and summarize the clinical and pathological features of patients with cryoglobulinemia. Methods A total of 16 patients diagnosed with cryoglobulinemia from April 2016 through December 2018 in Ruijin hospital affiliated to Shanghai Jiao Tong University School of Medicine were enrolled in the study. The clinical and laboratory data of patients and the renal pathological results were retrieved and analyzed. Results The patients investigated had an average age of(54.4 ±17.2) years, and 9(56.3%) were males. Four patients had hepatitis B virus(HBV) infection, 8 were with abnormal proliferation of lymphocyte and plasma cell, and 4 were complicated with au-toimmune diseases. 87.5%(14/16) of the patients had renal involvement, with renal function impairment identified in 10. Among 12 patients with renal biopsy performed, 7 were revealed to have membrano-proliferative glomerulonephritis(MPGN), 2 had tubulo-interstitial nephritis, 2 were with crescent nephritis and 1 presented thrombotic microangiopathy associated nephropathy. Seven patients needed plasma exchange in the treatment, 12 patients were placed on regimen of corticosteroid with or without immunosuppression, and 3 patients had been treated for the primary hematological malignancies. Within median 17-month-follow-ups, 3 patients died, 2 patients underwent maintenance hemodialysis,and all other patients maintained stable renal function. Conclusions Cryoglobulinemia and cryoglobulinemia associated renal lesion are not rare clinical conditions. The early diagnosis of cryoglobulinemia is critical due to its rapid progres-sion and relative poor prognosis.

关 键 词：冷球蛋白血症 临床特点 病理特征 预后 

分 类 号：R446[医药卫生—诊断学]