Castleman病肾损害的临床病理分析

目的 探讨Castleman病肾损害的临床病理特点。 方法 对10例Castleman病合并肾损害患者的临床病理资料进行分析。全部病例均接受了淋巴结和肾组织的病理检查，肾活检组织分别进行了光镜、免疫荧光和电镜检查。 结果 10例均为男性，平均年龄（49±14）岁。多数患者有水肿；全部患者均有蛋白尿，尿蛋白量（24 h）为（2.79±3.56）g，其中1例为肾病综合征（NS）；8例有血尿；6例并发急性肾功能不全；4例有高血压。多数有发热、乏力、纳差、体质量下降等。其他异常有贫血、血小板减少、浆膜腔积液、红细胞沉降率增块、高γ球蛋白血症、补体降低、C反应蛋白（CRP）升高、肝脾肿大、甲状腺功能低下等。2例符合POEMS综合征，1例有干燥综合征。10例均表现全身多处淋巴结肿大，以颈部、腋窝及腹股沟淋巴结肿大最常见。淋巴结活检病理类型分别为浆细胞型4例，透明血管型3例，混合型3例。肾活检病理诊断分别为血栓性微血管病5例、新月体性肾小球肾炎2例、肾淀粉样变1例、肾小球微小病变1例、慢性肾小管间质肾病1例。所有病例经过免疫抑制剂或COP方案治疗，病情均明显缓解，淋巴结缩小，蛋白尿减少或转阴，大部分病例肾功能恢复正常。 结论 Castleman病肾损害的临床和病理表现具有多样性，急性肾功能不全发生率较高，常并发全身多系统损害，肾脏病理以血栓性微血管病较多见。对于伴有全身多系统异常的肾脏病患者，有必要进行淋巴结影像学检查和淋巴结活检。

Clinicopathological analysis of Castleman disease with kidney injury

Objective To investigate the clinicopathological features of Castleman disease with kidney injury. Methods Clinicopathological data of 10 Castleman disease patients with kidney injury from Peking University First Hospital and China-Japan Friendship Hospital were analyzed retrospectively. All the cases received biopsies of lymph node and kidney. Their renal tissues were examined by light microscopy, immunofluorescence and electron microscopy. Results Ten patients were all male with mean age (49±14) years. They presented edema and proteinuria, with mean urinary protein at (2.79±3.56) g/24 h, including one nephrotic syndrome (NS). Hematuria occurred in 8 cases, acute renal insufficiency in 6 cases, hypertension in 4 cases. Most of the patients had fever, fatigue, anorexia, weight loss, increased ESR and CRP, hypergammaglobulinaemia and decreased complements. Other abnormalities included anemia, thrombocytopenia, pleural effusion, hepatomegaly, splenomegaly, hypothyroidism, etc. Two cases demonstrated POEMS syndrome, one presented Sjogren syndrome. The enlargement of multiple cervical, axillary and inguinal lymph nodes were identified in all the patients. The pathological patterns of lymph node were plasma cell type in 4 cases, hyaline-vascular type in 3 cases, and mixed type in 3 cases. Pathological examination of renal biopsy showed thrombotic microangiopathy in 5 cases, crescentic glomerulonephritis in 2 cases, renal amyloidosis, minimal change disease and chronic tubular interstitial nephropathy in 1 case respectively. After immunosupressive reagents or COP therapy, lymph nodes became smaller, systemic symptoms were alleviated, proteinuira was decreased or disappeared, and renal function was recovered in most of patients. Conclusions Castleman disease with kidney injury manifests various symptoms with high prevalence of renal insufficiency and multiple systemic damage. Renal lesions present many patterns of pathological change with a higher frequency of thrombotic microangiopathy. It is necessary to examine the lymph nodes by ultrasound, radiology or biopsy for the patients of renal diseases with multiple systemic symptoms.