合并肾损害的POEMS综合征患者的临床及预后分析

Analysis of clinicopathological characteristics and outcomes of patients with POEMS syndrome associated nephropathy

目的 分析合并肾损害的POEMS综合征患者的临床、肾脏病理特点及预后.方法 回顾性分析我院收治的6例POEMS综合征患者的临床表现、实验室检查、肾脏病理检查、治疗及随访等资料.结果 POEMS综合征男性多见,诊断时年龄28～65岁,平均（47.8±13.3）岁,诊断前病程平均4.1年.所有患者均有神经病变、脏器肿大、内分泌异常、皮肤改变,4例患者血M蛋白阳性,1例游离轻链比例异常.POEMS综合征肾损害多表现为轻中度蛋白尿,可合并肾功能不全,肾脏病理免疫荧光多阴性,或为免疫复合物的非特异沉积;光镜下肾小球和肾小动脉内皮细胞增生、肿胀,可合并缺血性肾损伤,透射电镜表现为内皮细胞增生、肿胀,基底膜内疏松层增厚,未见电子致密物.对于POEMS综合征的患者制定个体化治疗方案.随访平均25.8个月,1例合并Castleman病的患者死于感染合并多器官衰竭,其余5例病情稳定.结论 游离轻链比例异常可协助诊断POEMS综合征,本病的肾脏病理有特殊提示,肾活检为协助诊断本病的重要检查手段,单纯POEMS综合征的患者预后较好,合并Castleman病的患者预后相对较差.

Objective To analyze the clinicopathological characteristics and outcomes of patients with POEMS syndrome associated nephropathy. Methods Six consecutive patients diagnosed as POEMS syndrome in a single institute were retrospectively investigated. Among them, 3 patients were performed renal biopsy. Their clinical, pathological characteristics, treatment and outcomes were investigated. Results Males were more frequently involved than females in POEMS syndrome. The average age was 47.8 years old. The mean interval before diagnosis was 4.1 years. Every patient had neu-ropathy, organomegaly, endocrinopathy and skin changes. Four of them had monoclonal gammopathy, one had abnormal free light chain ratio. Patients had mild to medium urine protein excretion level with impaired renal function. Immunoglob- ulins and complements were negative or nonspecific deposit in immunofluorescent staining. Light microscopy showed en- dothelial cell proliferation and swelling of glomerular capillary and small arteries, lschemic lesions also presented. Trans- mission electron microscopy revealed endothelial cell proliferation and swelling with thickened lamina rara interna of glome,＇ular basement membrane. Individualized therapy was given to these patients. The average follow-up period was 25.8 months. One patient with Castleman disease died from infection and multi-organ failure, other 5 patients were in a stable condition. Conclusion Abnormal free light chain ratio is diagnostic. The renal pathology has specific manifestations which can be used as a diagnostic tool. Patients with simple POEMS syndrome have a good prognosis while patients with POEMS syndrome and Castleman disease may have worse prognosis.