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朗格汉斯细胞组织细胞增多症

Langerhans cell histiocytosis.

作者信息

Gasent Blesa Joan Manel, Alberola Candel Vicente, Solano Vercet Carlos, Laforga Canales Juan, Semler Christof, Pérez Antolí Maria Rosa, Rodríguez-Galindo Carlos

机构信息

Hospital General Universitari Marina Alta, Dènia, Alacant, Spain.

出版信息

Clin Transl Oncol. 2008 Nov;10(11):688-96. doi: 10.1007/s12094-008-0275-9.

Abstract

Langerhans cell histiocytosis (LCH) is a poorly understood proliferative disease, with different patterns of clinical presentation. Currently it is classified according to the number and type of system involved and the degree of organ dysfunction. The aetiology of the disease remains uncertain, and in some cases the disease is polyclonal, suggesting a reactive condition. Many cytokines have been implicated in the pathogenesis of LCH. Different therapeutic approaches can be considered depending on the affected organ, including surgery, radiotherapy and chemotherapy. Long-term organ dysfunction may remain, despite disease control and/or eradication, making indefinite supportive treatment mandatory. Here we present a literature review on all of the aspects of the disease, treatment approaches and existing protocols, and finally an adult clinical case.

摘要

朗格汉斯细胞组织细胞增多症(LCH)是一种了解甚少的增殖性疾病,具有不同的临床表现形式。目前,它是根据受累系统的数量和类型以及器官功能障碍的程度进行分类的。该疾病的病因仍不确定,在某些情况下,疾病是多克隆性的,提示一种反应性状态。许多细胞因子与LCH的发病机制有关。根据受累器官的不同,可以考虑不同的治疗方法,包括手术、放疗和化疗。尽管疾病得到控制和/或根除,但长期的器官功能障碍可能仍然存在,因此必须进行长期的支持治疗。在此,我们对该疾病的各个方面、治疗方法和现有方案进行文献综述,最后介绍一例成人临床病例。

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