中国人22例Castleman病的临床特点分析(英文)

Clinical characteristics of 22 cases of Castleman's disease in China

目的 Castleman病是一种少见的慢性淋巴组织增生性疾病 ,分析 2 2例确诊的Castleman病的临床特点 ,对其分类提出新见解。方法 采用病历回顾性分析方法。结果 局灶性Castleman病 12例 ,4例有干咳、腹痛等症状 ,病理均为透明血管型 (HV) ,手术切除预后好。单一部位肿物合并粘膜皮肤水疱和溃疡 6例 ,HV型 5例 ,混合型 (Mix) 1例 ,其中 5例出现呼吸困难 ,严重低氧血症 ,肺功能为严重阻塞性通气功能障碍 ,胸片双肺过度充气 ,CT可见弥漫性支气管扩张 ,临床符合闭塞性细支气管炎 ,治疗效果差。多中心性Castleman病 4例 ,浆细胞型 (PC) 3例 ,Mix型 1例。均有发热 ,3例浮肿、贫血、血沉加快和γ球蛋白增高 ,2例有肾功能不全 ,1例甲状腺功能减退 ,1例四肢远端神经病变 ,性激素水平异常 ,乳腺增生 ,诊断POEMS综合怔 ,予糖皮质激素、化疗等治疗 ,2例缓解 ,2例效果差 ,随访 3例患者死亡。结论 Castleman病临床病变多样 ,应重视皮肤黏膜型Castleman病。此型与多中心性Castleman均治疗困难。

Objective:Castleman's disease is a rare lymphoproliferative disorder that is often classified as hyaline vascular and plasma cell subtypes in histology. The aim was to investigate the clinical features of Castleman's diseases in China.Methods:22 cases of Castleman's diseases were analyzed retrospectively in the first Hospital of Peking University from 1977 to 2002.Results:Twelve patients with single mass had a good prognosis after operation. Only four of them had nonproductive cough and abdominal pain before operation. Six young patients were described with severe mucocutaneous ulceration and erosion. Tumors were found in the retroperitoneum and mediastinum, and pathological examination revealed Castleman's diseases (CD), in which five were hyaline-vascular type, and the other was mixed type. Five of them complained of progressive dyspnea with fatal outcome. The clinical features, radiological findings and the pulmonary function tests were consistent with those of bronchiolitis obliterans (BO). Four patients with multicentric CD presented manifestation of multisystem involvement. Three of them died although they had received corticosteroids, chemotherapy and radiation therapy.Conclusions:Symptoms of CD are diverse. Patients with mucocutaneous erosion frequently had lung involvement. It is different from traditional categories and should be considered as an independent type.