摘要目的 提高对累及气道的窦组织细胞增生症伴巨大淋巴结病(Rosai-Dorfman病)的认识.方法 分析1例确诊为气道受累的Rosai-Dorfman病患者的临床表现、支气管镜下表现、病理特征、诊断和治疗并结合文献进行讨论.结果 患者女,60岁.因"反复发作性喘息18个月,加重1个月"于2007年3月6日收入福建省立医院.曾诊断为"支气管哮喘",口服强的松、吸入布地奈德等治疗,症状好转.1个月前喘息再次发作,且出现吸气性呼吸困难,平卧位时明显,再次就诊,以"呼吸困难原因待查"收入院.23年前发现"高血压、左肾上腺腺瘤",诊断为"原发性醛固酮增多症",未行手术.1999年发现左腮腺肿物、左颌下淋巴结肿大,行手术切除.2000年行双眼泪腺肿物切除.2001年发现全身皮下多发性结节,且逐年增多,结节可自行增大和缩小.入院检查左颌下可触及大小约3.0 cm×2.0 cm结节,双上臂、胸背部、腹部、臀部及双大腿外侧可扪及黄豆至蚕豆大小结节,边界清楚,质偏硬,表面尚光滑,稍活动,轻压痛,部分皮肤见色素沉着.胸部CT平扫+气管重建显示气管壁多发性结节状突起,相应区域管腔狭窄,左肺舌叶炎症,纵隔内未见明显肿大的淋巴结,胸背部皮下多发性结节影.淋巴结病理切片可见组织细胞形成的浅染区与浆细胞和淋巴细胞形成的深染区相间排列,呈一种窦样结构,组织细胞胞质内可见吞噬的浆细胞和淋巴细胞,确诊为Rosai-Dorfman病.结论 Rosai-Dorfman病累及气道是罕见的淋巴结外病变,易发生误诊和漏诊.支气管镜检查是发现Rosai-Dorfman病气道受累的重要手段,组织细胞增生并吞噬完整的浆细胞和淋巴细胞是其病理特征,免疫组织化学染色S100蛋白及CD_(68)阳性有助于诊断和鉴别诊断.手术切除联合应用糖皮质激素或放疗是治疗气道病变较为有效的方法之一.

abstractsObjective To improve the awareness of sinus hisfiocytesis with massive lymphadenopathy (Rosai-Dorfman disease) involving the airways. Methods The clinical presentations, endoscopic findings in the airways, pathological characteristics, and diagnosis and treatment of a case of Resai-Dorfman disease was reported, and related literatures were reviewed. Results A 60-year-old female patient was admitted to this hospital because of recurrent wheezing for 18 months and aggravated for 1 month on March 6, 2007. The diagnosis of "bronchial asthma" had been made and oral predniselone and inhaled budesonide resulted in symptom improvement. One month ago, she had wheezing again with inspiratory dyspnea, which was more obvious at recumbent position. She had been found to have high blood pressureand left adrenal adenoma 23 years ago, and as the diagnosis of "primary aldosteronism" was made but underwent no surgery. Left parotid gland tumor and left submandibular lymph nodes had been found, and surgical resection implemented in 1999. Lacrimal gland tumor resection of her eyes had been performed in 2000. Multiple subcutaneous nodules, rising and disappearing spontaneously, had been demonstrated in 2001. After admission, physical examination revealed nodules of 3.0 cm×2.0 cm in her left submandibular area, and soybean sized nodules atboth arms, back, chest, abdomen, buttocks and thighs. Chest CT scan and tracheal reconstruction showed that there were multiple nodules in the tracheal wall with narrow lumen, with no obvious enlargement of mediastinal lymph nodes. Lymph node biopsy showed faintly stained areas and the formation of plasma cells and lymphocytes of the deeply stained area, presenting as a sinus-like structure, and plasma cells and lymphocytes were engulfed in the plasma of the histiocytes, consistent with the diagnosis of Rosai-Dorfman disease. Conclusions Rosai-Dorfman disease involving the airway was a rare disease often misdiagnosed. Bronchoscopy was very helpful for the dianosis. Histiocytosis with phagocytosis of plasma cells and lymphocytes was the pathological feature, and immunohistochemical staining positive for S100 protein and CD_(68) was suggestive of the diagnosis. Surgical resection combined with corticosteroids or radiotherapy was effective treatment of the airway diseases.