胸部受累的巨淋巴结增生症10例临床分析

Thoracic involvement of Castleman＇ s disease： report of 10 cases

目的提高对少见胸部受累的巨淋巴结增生症的认识，减少误诊，延长患者的生存期。方法2000年1月至2006年5月对10例胸部受累的巨淋巴结增生症患者的临床表现、影像学、病理学、鉴别诊断、治疗及预后进行研究。结果10例胸部受累的巨淋巴结增生症患者均为女性，其中5例多中心型患者的年龄为29—49岁，临床表现多样，外周淋巴结多处肿大伴多系统损害，影像学表现为间质性肺炎和纵隔淋巴结肿大，病理分型均为浆细胞型，给予糖皮质激素或化疗后4例部分缓解，1例死于心肺衰竭。5例局灶型患者的年龄为13—49岁，均无症状体征，实验室检查无异常，病灶均位于右侧纵隔，直径6—9cm，病灶呈均匀显著强化，病理分型均为透明血管型，经手术切除肿块，5例随访均存活。结论胸部受累的巨淋巴结增生症易误诊，有多发淋巴结肿大或局灶巨大淋巴结者应警惕该病的发生。诊断的关键在于早期多次、多部位淋巴结病理检查，与血管同步显著强化的影像学表现具有诊断价值。

Objective To improve the recognition of thoracic involvement of Castleman＇ s disease Methods Ten patients with thoracic involvement of Castleman＇ s disease were retrospectively studied by review of the clinical manifestations, radiology, pathology, differential diagnosis, therapy and prognosis. Results The 10 patients were all females. Five of the patients, aged from 29 to 49 years, presented with multicentric lesions, manifested by interstitial pneumonia and mediastinal lymphadenopathy on radiology, as well as extrathoracic involvement including peripheral lymphadenopathy, and multiple organ impairment. The pathology was the plasma cell type. Treatment with corticosteroids and chemotherapy achieved partial remission in 4 cases, but 1 died of cardiac and respiratory failure. Localized lesions were found in the other 5 patients, aged from 13 to 49 years. No specific manifestations were revealed in these patients. The major radiological sign was right mediastinal masses, 6 -9 cm in diameter, unifomly enhanced by contrast radiology. The pathology revealed vascular hyaline degeneration. Early surgical resection achieved good prognosis. The 5 patients were all alive. Conclusions Diagnosis of thoracic involvement of Castleman＇ s disease is not easy. Early, repeated and multiple biopsy of lymph nodes is recommended, especially when the disease is muhicentric or when the lymph nodes are massive. Synchronous enhancement with the vessels on contrast radiology is suggestive of the diagnosis.