淋巴瘤样肉芽肿与韦格内肉芽肿临床特点的比较

Dinfferentiation between lymphomatoid granulomatosis and Wegener granulomatsis

目的 总结淋巴瘤样肉芽肿病(lym phom atoid granulom atosis,LG)与韦格内肉芽肿( egener granulo Wm atosis,W G)的临床特点,从临床上加以鉴别。 方法 回顾性总结我院与北京协和医院 1989 年以来收治的 6 例 LG和20 例 W G 患者的临床资料,并进行比较。 结果 发热、咳嗽及呼吸困难在 LG 中较常见,流涕、鼻衄及关节痛以W G 为多,肺受累以 LG 为多,耳鼻喉、眼及肾病变在 W G 中明显增多,皮肤受损两者相似。W G 患者常有 c-ANCA 阳性及尿沉渣异常。 LG X 线胸片为双侧多发结节,边界不清,多无肺门、纵隔淋巴结肿大; G 则为双侧多发边界清晰W的结节, 易形成空洞, 肺门和纵隔淋巴结可肿大。LG 病理改变为血管中心性淋巴增生性病变, 浸润的细胞主要是小淋巴细胞和不同数量大的不典型淋巴细胞; 而在 W G 中可见坏死性血管炎及大量中性白细胞、浆细胞及少量嗜酸细胞浸润形成的肉芽肿, 部分有多核巨细胞。W G 经治疗后, 多数患者病情缓解; 则疗效不佳。 结论 LG 和 W G 在临 LG床表现、实验室检查、 影像及病理诸方面的差异有利于两者的鉴别诊断。

Objective To differentiate clinical featuers of Lymphomatoid granulomatosis(LG) and Wegener granulomatosis(WG). Methods The clinical manifestations of 6 patients with LG and 20 patients with WG admitted to Tianjin First Central Hospital and Peking Union Medical College (PUMC) Hospital since 1989 were retrospectively analyzed. Results ① Lung damage: LG was more than WG.② Skin involvemert: both were similar.③ Ear-nose-throat?eye and kidney involvemert: WG was more than LG. ④ Clinical manifestations: fever, cough and dyspnea were similar in LG and WG nasal obstruction, purulent nasal discharge, nasal hemorrhage,paranasal sinusitis and arthralgia were more in WG than in LG. ⑤ Laboratory examinations : c-ANCA positive and abnormal urine routine were aften shown in WG. ⑥ Pathological findings: an angiocentric and angiodystructive lymphoproliferative syndrome were observed in LG. Exudation; necrosis; lots of neutrophil leukocytes, plasmocytes and a few acidophil infiltration and granulation could be found in WG. ⑦ Therapy and prognosis: 2 LG patients were improved and 2 died. 16 WG patients survived but 4 died. Conclusions LG and WG are different in clinical manifestations, organs involvement, laboratory pathological examinations and X-ray features.