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

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

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＆#177;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。

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 ana-lyzed their clinical menifestations, diagnosis, treatment, and prognosis. Results There were 4 males and 2 fe-males in the 6 patients, whose ages of onset were 9-24 years with a mean age of （16. 1 ＆#177; 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 ca-ses 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.