成人朗格汉斯细胞组织细胞增生症的诊疗

朗格汉斯细胞组织细胞增生症(LCH)是一种以MAPK信号通路激活为主要特征的克隆性血液系统肿瘤,属于炎性髓系肿瘤。其临床表现多样,最常见的临床受累脏器为骨骼、肺、垂体等。其主要依赖于病理活检确诊,病理免疫组化CD207和电镜检查可见伯贝克颗粒是诊断的金标准。根据临床受累脏器范围,LCH分为单系统单病灶、单系统多病灶、多系统受累。治疗主要根据临床分型,对于单系统单病灶患者以局部治疗为主,对于单系统多病灶或多系统LCH以全身治疗为主。

Diagnosis and treatment of adult Langerhans cell histiocytosis

Langerhans cell histiocytosis(LCH) is an inflammatory myeloid malignancies with MAPK pathway activation mutations detectable in nearly all patients and a pronounced inflammatory state. The clinical manifestations of LCH are diversity, the most common involved organs included Bones, lungs, pituitary, ect. The primary diagnosis of LCH depends on histopathologic review of lesional tissue biopsy; the gold standard for diagnosis were expression of Cd207 (Langerlin) in immunohistochemistry and birbeck granules(BGs) by electron microscopy. LCH can be devided into localized single-system disease(SS-s), multiple sites within one single system (SS-m), and multisystem(MS) disease according to the scope of clinically involved organs. In terms of clinical classification, Local therapies may be sufficient for SS-s patients, systemic therapies might be better for SS-m or MS patients.