Division of Pediatrics, Takasago-seibu Hospital, 1-10-41 Nakasuji, Takasago 676-0812, Japan.
Int J Hematol. 2011 Dec;94(6):556-60. doi: 10.1007/s12185-011-0955-z. Epub 2011 Oct 21.
We report four cases of adult-onset Langerhans cell histiocytosis (LCH) with central nervous system (CNS) lesions in the hypothalamic-pituitary region. The first clinical symptoms were diabetes insipidus (two patients), hypothyroidism (one patient), and decreased libido/erectile dysfunction (one patient). Diagnosis was delayed as the CNS lesion was not initially suspected to be secondary to LCH, with a median time from symptom onset to treatment of 3.0 (range <1-5.3) years. In three patients, the tumor mass was effectively reduced by chemotherapy; however, all patients continue to exhibit hypopituitarism. Early diagnosis and initiation of treatment are required to improve the outcome of CNS-LCH in adult patients.
我们报告了 4 例成人发病的朗格汉斯细胞组织细胞增生症（LCH）伴下丘脑-垂体区域中枢神经系统（CNS）病变。首发临床症状为尿崩症（2 例）、甲状腺功能减退症（1 例）和性欲减退/勃起功能障碍（1 例）。由于最初并未怀疑 CNS 病变是由 LCH 引起的，因此诊断被延误，从症状出现到治疗的中位时间为 3.0 年（范围<1-5.3 年）。在 3 例患者中，肿瘤体积通过化疗得到有效缩小；然而，所有患者仍存在垂体功能减退。为改善成人 CNS-LCH 的预后，需要做到早期诊断和及时开始治疗。
