华氏巨球蛋白血症相关肾损害的临床病理特征

Clinical and pathological features of renal disease associated with Waldenstrom macroglobulinemia

目的:了解华氏巨球蛋白血症(WM)相关肾脏损害的临床和病理特征.方法:回顾分析2002年2月~2019年9月在国家肾脏疾病临床医学研究中心因肾脏损害入院的WM患者的临床表现、实验室检查及肾活检病理.结果:(1)一般情况:本研究共纳入16例患者,平均年龄60.9±11.3岁,男性11例,女性5例,WM病程3(1~6.75)个月.(2)临床特征:11例首发肾脏损害表现为肾功能不全;就诊时中位尿蛋白定量1.79g/24h(0.75~2.97 g/24h);血清肌酐183.8μmol/L(76.9~359.7μmol/L).16例均有贫血和血清IgM均升高,12/16例球蛋白升高,11例免疫固定电泳示IgM单克隆免疫球蛋白条带,15/15例血轻链比值异常,4/11例血冷球蛋白阳性,8/14例低补体血症;2/15例尿本周蛋白阳性;3/16例脾肿大.(3)病理特征:直肠淀粉样变性3例(1例合并皮肤淀粉样变性).8例行肾活检,3例肾小球系膜增生病变;肾小球轻微病变、肾小球内皮细胞病变、肾小球膜增生样病变、肾小球毛细血管袢皱缩、κ型轻链沉积病各1例.(4)治疗及预后:11例明确诊断后接受化疗,3例接受免疫吸附.6例死亡,8例存活(其中1例转化为骨髓瘤,4例缓解)2例失访.结论:WM相关肾损害好发于中老年,男性多见,肾功能不全发生率高,肾脏病理改变多种多样,总体预后不佳.

Objective:To investigate clinical and pathological characters of renal disease associated with Waldenstrom macroglobulinemia(WM).Methodology:Clinical features,laboratory and pathological data of WM patients with renal involvement recruited from Jan 2002 to September 2019 were retrospectively analyzed.Results:16(11 males and 5 females)patients were included with a mean age of 60.9±11.3 years old.Renal dysfunction was the initial presentation in 11 patients,serum creatinine(SCr)was 183.8(76.9~359.7)μmol/L.IgM elevated in all 16 cases,of which 12 cases had hyperglobulinemia.Immunofixation electrophoresis showed monoclonal IgM in 11 cases,abnormal free light chain ratio in 15 cases,anemia in 16 cases.Rectal mucosa amyloidosis were proved in 3 cases.8 patients underwent renal biopsy,3 of them were diagnosed as glomeruli mesangial hyperplasia,while others were diagnosed minimal change of glomeruli,glomerular endothelial change,membranousproliferative glomerular nephritis,glomerular capillary loop wrinkle,κ-light chain deposition disease separately.11 patients received chemotherapy after diagnosis,3 patients received immunoadsorption.6 patients died,8 survived and 2 patients lost follow-up.Among the survived patients,1 patient advanced to multiple myeloma and 4 patients got remission.Conclusion:Renal disease associated with WM mostly occur in middle-aged males,with a high incidence of renal insufficiency and various renal pathological findings.The prognosis of these patients was poor according to our analysis.