目的 探讨肺郎格罕细胞组织细胞增多症（pulmonary Langerhans cell histiocytosis, PLCH）的临床特征、诊断和治疗。 方法 回顾性分析1999年-2008年4例病理确诊的PLCH。 结果 4例患者均为男性；13～56岁；2例吸烟。临床特征是活动后气短、气胸。胸部CT表现为网格状、囊状或结节状影像。肺活检病理结果：光学显微镜下可见病理性郎格罕细胞，免疫组织化学法检测发现4例S-100均为阳性，2例CD1a阳性，1例CD68阳性。 结论 PLCH胸部CT表现为网结节或囊性变，病理检查见病理性郎格罕细胞浸润细支气管壁和上皮细胞的间质，免疫组织化学CD1a抗原、S-100蛋白阳性可明确诊断。
目的:探讨郎罕氏组织细胞增多症累及甲状腺的临床表现,诊治及预后。方法: 淋巴结病理活检及免疫组化证实郎罕氏组织细胞增多症的诊断,颈部血管彩超发现甲状腺长大。予以小剂量化疗。结果: 2例患者均为系统性郎罕氏组织细胞增多症,累及甲状腺,予以小剂量化疗后,其中1例患者,化疗后淋巴结及甲状腺明显缩小。另1例患者广泛受累,化疗后效果差。结论: 郎罕氏组织细胞增多症累及甲状腺者罕见,可表现为甲状腺单独受累或伴多系统受累,对前者可采用甲状腺部分或全切术,疗效好;对于多器官受累者多采用联合化疗,但疗效较差。
ObjectiveTo investigate the clinical, radiographic characteristics and differential diagnosis of pulmonary Langerhans cell histiocytosis (PLCH) mimicking metastasis of cancer in radiography. MethodsClinical data of 2 patients with PLCH manifesting as metastatic cancer on HRCT and PET/CT were retrospectively analyzed. Patients reported as PLCH on WanFang Database, China Knowledge Resource Integrated Database and Pubmed were reviewed to screen misdiagnosis literature and further analyzed the clinical and radiographic characteristics. ResultsTwo cases both presented with cough and sputum. 18F-FDG PET/CT showed increased 18F-FDG up-take in both nodules in the lungs. One patient presented with multiple nodules, diffuse multiple cystic changes in lungs and osteoclasia in the right 4th rib on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of rib biopsy. The other patient presented with diffuse multiple nodules on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of lung biopsy. The pathological characteristics of both biopsy specimen demonstrated infiltration by Langerhans cells (LC) and eosinophils. The LC were positive for CD1a. Literature review found seven PLCH cases who were misdignosed as depression, eosinophilic pneumonia, interstitial lung disease involvement of autoimmune disorders and malignant tumor. ConclusionWhen clinician faced with a patient suspected as metastatic cancer by HRCT and PET/CT, it is reasonable to consider PLCH as a differential diagnosis and obtain the pathological information as soon as possible so that better prognosis can be achieved through early intervention.