累及甲状腺的朗格汉斯组织细胞增生症6例报道及文献复习

  目的  探讨累及甲状腺的朗格汉斯组织细胞增生症(Langerhans' cell histocytosis, LCH)的临床特点。  方法  回顾性分析2004年1月至2014年12月北京协和医院明确诊断有甲状腺受累的6例LCH患者的临床资料并总结其临床表现、诊断、治疗和预后情况。  结果  6例患者中男性4例, 女性2例, 起病年龄9~24岁, 平均年龄(16.1±5.7)岁; 6例患者均有垂体后叶受累, 4例有肺脏受累, 3例有肝脏受累; 4例患者甲状腺自身抗体阴性, 2例阳性; 4例患者通过甲状腺粗针穿刺活检获得病理证实; 5例患者采用化疗方案治疗; 随访4例患者中3例病情稳定无进展。  结论  在有多器官受累的甲状腺肿大患者中, 需进一步排除LCH甲状腺受累的可能性, 必要时进行甲状腺穿刺, 特别是粗针穿刺以明确病理诊断, 同时在LCH甲状腺受累患者中要特别注意有无肝脏受累。

Langerhans' Cell Histocytosis with Thyroid Involvement: Report of Six Cases and Literature Review

Objective　To investigate the clinical characteristics of Langerhans cell histocytosis (LCH) involving the thyroid gland. Methods　We reviewed 6 cases of histologically confirmed LCH with thyroid gland involvement in Peking Union Medical College Hospital in the period of January 2004 to December 2014 and analyzed their clinical menifestations, diagnosis, treatment, and prognosis. Results　There were 4 males and 2 females in the 6 patients, whose ages of onset were 9-24 years with a mean age of (16.1±5.7) years. All the 6 cases had posterior pituitary involvement, 4 cases had lung involvement, and 3 cases had liver involvement. There were 4 cases with negative thyroid autoantibodies and 2 cases with positive thyroid autoantibodies. Four cases were pathologically confirmed by core needle aspiration biopsy. Five cases were treated with chemotherapy. In the 4 cases followed up, 3 were in progress free stable status. Conclusions　LCH with thyroid involvement should be considered in thyromegaly patients with multiple organs involvement. Thyroid aspiration, especially core needle aspiration biopsy, is required to confirm pathological diagnosis if necessary. Special attention should be paid to detect liver involvement in patients with thyroid LCH.