Azreen A B, Kwan C Y, Prepagaren N
University of Malaya, Faculty of Medicine, 50603 Kuala Lumpur, Malaysia.
Med J Malaysia. 2012 Dec;67(6):627-8.
Langerhan cell histiocytosis is a rare disease and usually occurs in paediatric age group. This disease may involve single or multiple organs system and has an unpredictable course of disease. The involvement of head and neck region are almost 90% of cases, however maxillary sinus involvement is very rare. We report a case of 2 year old boy presented with multi organ LCH (orbit, skull, sinus and liver). The mainstay treatment for this high risk multi organ LCH group is chemotherapy. Unfortunately, although with the advancement of treatment, their mortality rate is still high.
朗格汉斯细胞组织细胞增多症是一种罕见疾病，通常发生于儿童年龄组。这种疾病可能累及单个或多个器官系统，且疾病进程不可预测。头颈部受累的病例几乎占90%，然而上颌窦受累非常罕见。我们报告一例2岁男孩，患有多器官朗格汉斯细胞组织细胞增多症（累及眼眶、颅骨、鼻窦和肝脏）。对于这种高危多器官朗格汉斯细胞组织细胞增多症组，主要治疗方法是化疗。不幸的是，尽管治疗有所进展，但其死亡率仍然很高。
