Lourenço Jorge, Ferreira Cristina, Marado Daniela
Department of Internal Medicine, Coimbra University Central Hospital, 3000-075 Coimbra, Portugal.
Department of Pneumology, Coimbra University Central Hospital, 3000-075 Coimbra, Portugal.
Mol Clin Oncol. 2020 Oct;13(4):30. doi: 10.3892/mco.2020.2100. Epub 2020 Jul 22.
Langerhans cell histiocytosis (LCH) is a rare systemic and heterogeneous disease secondary to proliferation and diffuse infiltration of immature CD1a-positive dendritic cells, also known as Langerhans cells. LCH affects predominantly paediatric patients and is rarely diagnosed in adulthood. Despite its worldwide prevalence, most reported cases are found in the Japanese population. There is no consensus regarding treatment strategy due to the low incidence of this disease and the diversity of symptoms that appear. An integrative literature review was conducted based on the PubMed database using MeSH terms 'Langerhans', 'histiocytosis' and 'adult'. The present report describes a case of a successfully treated LCH-induced central diabetes insipidus (uncommon presentation in adult patients) as well as an updated review of current evidence published on this matter.
朗格汉斯细胞组织细胞增多症(LCH)是一种罕见的系统性异质性疾病,继发于未成熟的CD1a阳性树突状细胞(也称为朗格汉斯细胞)的增殖和弥漫性浸润。LCH主要影响儿童患者,在成年期很少被诊断出来。尽管其在全球范围内都有发生,但大多数报告病例都出现在日本人群中。由于这种疾病的发病率低且出现的症状多样,因此关于治疗策略尚无共识。使用医学主题词“朗格汉斯”、“组织细胞增多症”和“成人”,基于PubMed数据库进行了综合文献综述。本报告描述了一例成功治疗的LCH诱发的中枢性尿崩症(成年患者中不常见的表现)病例,以及对此事已发表的当前证据的最新综述。