成人朗格汉斯细胞组织细胞增生症的诊疗

Diagnosis and treatment of adult Langerhans cell histiocytosis

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。

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.