Skowronska-Jozwiak Elzbieta, Sporny Stanislaw, Szymanska-Duda Joanna, Baranska Dobromila, Lewinski Andrzej
Department of Endocrinology and Metabolic Diseases, Medical University of Lodz, Poland.
Polish Mother's Memorial Hospital Research Institute, Lodz, Poland.
Neuro Endocrinol Lett. 2016 Jul;37(3):174-178.
Langerhans cell histiocytosis (LCH) in adults is a rare disorder of unknown etiology characterized by monoclonal proliferation of Langerhans cells. It belongs to dendritic cell disorders and occurs in 1-2 adults per million. The most common endocrine manifestation of classical LCH is associated with the posterior pituitary, with clinical symptoms of diabetes insipidus. Less than 80 reported cases of LCH involving the thyroid gland have been published so far. We present the case of a 39 years old woman with 10 years history of diabetes insipidus and secondary amenorrhoea, which appeared after second delivery. She was suspected for lymphocytic inflammation of pituitary and she was administered steroid treatment. She was also treated symptomatically with desmopressin, L-thyroxine, estrogen and progestagen replacement therapy due to diabetes insipidus, secondary hypothyroidism and hypogonadotropic hypogonadism. In September 2014, she noticed a painless, firm tumour of the neck. Ultrasound (US) examination demonstrated bilateral, solid, hypoechogenic thyroid nodules. The result of fine-needle aspiration biopsy (FNAB) was not diagnostic. Due to rapid progression and US image of the tumour, she was referred for surgery. In postoperative histopathology tumour cells were positive for CD1a and S-100 protein, therefore diagnosis of LCH was established. Postoperatively, the results of thoracic computed tomography scan, abdominal US and bone scintigraphy revealed no evidence of multifocal disease. We have not observed any disease recurrence in the patient after a year of follow-up in postoperative course. This case illustrates diagnostic and therapeutic difficulties in patient with LCH.
成人朗格汉斯细胞组织细胞增多症（LCH）是一种病因不明的罕见疾病，其特征为朗格汉斯细胞的单克隆增殖。它属于树突状细胞疾病，每百万成年人中发病率为1至2例。经典LCH最常见的内分泌表现与垂体后叶有关，临床症状为尿崩症。迄今为止，已发表的涉及甲状腺的LCH病例报道不足80例。我们报告一例39岁女性病例，她在第二次分娩后出现了10年的尿崩症和继发性闭经病史。她曾被怀疑患有垂体淋巴细胞性炎症，并接受了类固醇治疗。由于尿崩症、继发性甲状腺功能减退和低促性腺激素性性腺功能减退，她还接受了去氨加压素、左甲状腺素、雌激素和孕激素替代疗法的对症治疗。2014年9月，她注意到颈部有一个无痛性、质地坚硬的肿块。超声（US）检查显示双侧实性低回声甲状腺结节。细针穿刺活检（FNAB）结果无法确诊。由于肿瘤进展迅速及超声图像表现，她被转诊接受手术。术后组织病理学检查显示肿瘤细胞CD1a和S-100蛋白呈阳性，因此确诊为LCH。术后，胸部计算机断层扫描、腹部超声和骨闪烁显像结果均未发现多灶性疾病的证据。术后随访一年，我们未观察到该患者有任何疾病复发情况。本病例说明了LCH患者诊断和治疗方面的困难。
