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目的 探讨Castleman病 (CD) ,即血管滤泡性淋巴结增生 (ALNH)的临床表现及病理学特点。方法 回顾 6例CD的临床表现、淋巴结及神经活检等。结果 多中心型CD 4例 ,均有系统症状 ,如发热、消瘦、贫血、水肿 ,内分泌和代谢异常等 ;3例检出单克隆蛋白 :3例多中心型合并周围神经病 ,表现为慢性对称性四肢无力、麻木 ,肌力减低 ,腱反射消失 ,对称性远端感觉减退。电生理检查提示运动和感觉神经动作电位波幅减低 ,腓肠神经活检 2例 ,显示轴索变性、脱失 ,以大有髓纤维受累为主。局灶型CD 2例 ,无系统症状或周围神经病表现。 6例经病理学检查确诊CD ,5例为透明血管型 ,1例为浆细胞型。结论 多中心型CD可表现为多系统受累和周围神经病 ,符合POMES综合征 ,其周围神经病可表现为远端对称性感觉运动性轴索性神经病
Objective To investigate the clinical manifestations and pathological features of Castleman’s disease (CD), the vascular hyperplasia of the follicular lymph nodes (ALNH). Methods The clinical manifestations, lymph nodes and nerve biopsy of 6 cases of CD were reviewed. Results There were 4 cases of multicentric CD with systemic symptoms such as fever, weight loss, anemia, edema, endocrine and metabolic abnormalities. 3 cases were detected monoclonal protein: 3 cases of polycentric central neuropathy with chronic symmetry Limbs weakness, numbness, decreased muscle strength, tendon reflex disappeared, the symmetry of distal decline. Electrophysiological examination showed that the amplitude of motor and sensory nerve action potentials decreased. Two cases of sural nerve biopsy showed degeneration and loss of axon. Focal CD 2 cases, no symptoms or peripheral neuropathic performance. 6 cases were diagnosed by pathological examination of CD, 5 cases of transparent vascular type, 1 case of plasma cell type. Conclusions Multicentric CD can manifest as multisystem involvement and peripheral neuropathy, accord with POMES syndrome. Its peripheral neuropathy can be characterized by distal symmetrical sensory motor axonal neuropathy