郎格罕细胞组织细胞增生症的诊治(附24例报告)

Diagnosis and Treatment of Langerhans Cell Histiocytosis (Clinical Analysis of 24 Cases)

目的:总结郎格罕细胞组织细胞增生症(LCH)的诊治经验。方法:对24例郎格罕细胞组织细胞增生症临床资料进行分析。结果:诊断为勒雪氏病(LS)16例,韩雪柯氏综合征(HSC)2例,骨嗜酸性肉芽肿(EGB)4例,难分型者2例。同时具临床、X线片和病理诊断者16例(占667%),诊断后给予放、化疗。3例死亡,5例放弃治疗,7例好转出院后失访,4例自动出院,5例坚持治疗至疗程结束,随访至今无复发。结论:重视临床表现尤其特征性皮疹并常规做皮疹印片,充分认识X线典型改变,有助于早期诊断;早期、联合、足量化疗辅以手术、放疗可改善预后。VEP(V为长春新碱,E为足叶乙甙,P为强的松)方案疗效好,副反应轻。

Objective: To discuss the diagnosis and treatment of Langerhans Cell histiocytosis (LCH). Methods: Clinical analysis was performed in 24 cases with LCH.Results: Among all the cases, 16 cases were Lettere-Siwe disease (LS), 2 Hand-Schuller-Chistian syndrome (HSC), 4 eosinophilic granuloma of bone (EGB) and the other 2 difficult to be typed. Sixteen cases were diagnosed by clinical manifestations, X-ray and pathologic changes. All the patients were treated with radiotherapy and chemotherapy and 3 died, 5 gave up treatment at the beginning, 7 discharged with better condition but lost contact then, 4 left hospital with no improvement, only 5 held on to the end of the therapy period and the following-up showed no recurrence. Conclusion: Attention should be paid to clinical manifestations, especially the special skin rash, and the skin print should be done routinely. Fully understanding of the typical X-ray changes will do good to early diagnosis. Early combined fulldose chemotherapy, radiotherapy and operation treatment can improve the prognosis. Chemotherapy composed of Vincristin, Etoposide and Predison (VEP) is effective and has light side-effects.