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Langerhans' Cell Histocytosis with Thyroid Involvement: Report of Six Cases and Literature Review
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摘要:
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。 -
关键词:
- 朗格汉斯组织细胞增生症 /
- 甲状腺疾病
Abstract: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. -
Key words:
- Langerhans' cell histocytosis /
- thyroid disease
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表 1 6例朗格汉斯组织细胞增生症甲状腺受累患者临床资料
病例 性别 起病年龄
(岁)病程
(年)首发症状 其他症状 受累脏器 甲状腺超声表现 甲状腺功能 甲状腺自身
抗体(U/ml)活检或手术部位 活检病理免疫组化 治疗方案 预后 病例1 6 13 男 尿崩 颈部增粗、自发气胸 垂体后叶、甲状腺、肺 甲状腺弥漫性病变伴多发结
节，甲状腺肿大继发性甲减 TGAb＞1000，
TPOAb 39.5甲状腺粗针穿刺活检 CD1α、S-100(+) COEP×1、MTX×1、
COEP×3随访5年后无明显进展 病例2 男 20 36 口腔溃疡 牙齿脱落、皮疹、一过性
尿崩皮肤黏膜、垂体后叶、甲状
腺、肺甲状腺弥漫性病变 原发性甲减 阴性 口腔黏膜活检 CD1α、S-100(+) COEP×4、CHOEP×3 随访半年后症状明显好转 病例3 女 12 7 尿崩 颈部增粗、腹水 垂体后叶、下丘脑、甲状腺、
肺、肝甲状腺弥漫性肿大 原发性甲减 阴性 甲状腺细针穿刺活检阴性，后取甲状腺手术病理 CD1α、S-100(+) 甲状腺部分切除、
COEP×3、FND×2随访3年后病情进展，出现下丘脑综合征 病例4 男 9 2.5 尿崩 颈部增粗 垂体后叶、甲状腺、肺 甲状腺弥漫性病变伴肿大 亚临床甲减 阴性 甲状腺粗针穿刺活检 CD1α、S-100(+) MVP×40 随访1年垂体柄及甲状腺缩
小，无进展，无新发病变病例5 女 24 2 尿崩 间断发热、皮疹、颈部增粗 垂体后叶、皮肤、甲状腺、肝 甲状腺弥漫性病变伴肿大 原发性甲减 TGAb阴性，
TPOAb 45.0甲状腺粗针穿刺活检 不详 COEP×1 不详 病例6 男 19 2 肝功能异常 尿崩、颈部增粗 肝、垂体后叶、甲状腺 甲状腺弥漫性病变伴肿大 亚临床甲减 阴性 甲状腺粗针穿刺活检 CD1α、S-100(+) 治疗方案不详 不详 TGAb：甲状腺球蛋白抗体；TPOAb：甲状腺过氧化物酶抗体；COEP：环磷酰胺、长春地辛、依托泊苷、泼尼松；MTX：氨甲蝶呤；CHOEP：环磷酰胺、依托泊苷、长春地辛、表柔比星、泼尼松；FND：氟达拉滨、米托蒽醌、地塞米松；MVP：氨甲蝶呤、长春地辛、泼尼松 
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