Herzog K M, Tubbs R R
Cleveland Clinic Foundation, Ohio, USA.
Adv Anat Pathol. 1998 Nov;5(6):347-58. doi: 10.1097/00125480-199811000-00001.
Langerhans cell histiocytosis (LCH) is a rare clonal disorder that consists of single or multiple mass lesions composed of cells with an abnormal Langerhans cell phenotype. Its etiology remains unknown, despite extensive searches for evidence of consistent cytogenetic abnormalities, gene rearrangements, or viral genomes. Similarly, the pathogenesis of the disease is enigmatic, although the altered expression of cytokines and cellular adhesion molecules, important for migration and homing of the activated normal Langerhans cell, may play an important role. The biologic behavior of LCH ranges from spontaneous remission to lethal dissemination, and such behavior cannot be predicted on the basis of histologic features. The presence and degree of organ dysfunction, together with the patient's age at diagnosis, remain the most reliable indicators of prognosis. Treatment of severe, refractory disease continues to be controversial and, in many cases, ineffectual. The revised classification scheme for LCH and related disorders recognizes the uncertain biological potential of LCH and its relation to other processes of dendritic and macrophage origin.
朗格汉斯细胞组织细胞增多症（LCH）是一种罕见的克隆性疾病，由单个或多个由具有异常朗格汉斯细胞表型的细胞组成的肿块性病变构成。尽管广泛寻找一致的细胞遗传学异常、基因重排或病毒基因组的证据，但其病因仍不清楚。同样，尽管对激活的正常朗格汉斯细胞的迁移和归巢很重要的细胞因子和细胞黏附分子的表达改变可能起重要作用，但该疾病的发病机制仍不清楚。LCH的生物学行为从自发缓解到致死性播散不等，且这种行为无法根据组织学特征预测。器官功能障碍的存在和程度，以及诊断时患者的年龄，仍然是最可靠的预后指标。严重难治性疾病的治疗仍然存在争议，而且在许多情况下是无效的。LCH及相关疾病的修订分类方案认识到LCH的生物学潜能不确定及其与其他树突状细胞和巨噬细胞起源过程的关系。
