Harmon Charles M, Brown Noah
From the Department of Pathology, University of Michigan Hospital and Health Systems, Ann Arbor.
Arch Pathol Lab Med. 2015 Oct;139(10):1211-4. doi: 10.5858/arpa.2015-0199-RA.
Langerhans cell histiocytosis (LCH) comprises a wide spectrum of clinical disorders that have in common a proliferation of Langerhans-type cells with characteristic morphologic, immunophenotypic, and ultrastructural features. In part because of the diverse clinical manifestations of LCH, there has long been controversy over whether LCH is best considered a reactive process or a neoplasm. Herein, we discuss the clinical and pathologic features of LCH, including recent advances in the understanding of the molecular pathogenesis of this disease that support its categorization as a neoplasm. We also review the implications that these recently described molecular characteristics may have on risk stratification and treatment of LCH.
朗格汉斯细胞组织细胞增多症（LCH）包括一系列临床病症，其共同特征是具有特征性形态、免疫表型和超微结构特征的朗格汉斯型细胞增殖。部分由于LCH临床表现多样，长期以来关于LCH最好被视为反应性过程还是肿瘤存在争议。在此，我们讨论LCH的临床和病理特征，包括对该疾病分子发病机制理解的最新进展，这些进展支持将其归类为肿瘤。我们还回顾了这些最近描述的分子特征可能对LCH的风险分层和治疗产生的影响。
