郎格罕氏组织细胞增生症的诊断与治疗

Diagnosis and Therapy of Langerhans Cell Histocytosis

目的:总结郎格罕氏组织细胞增生症(LCH)的诊断与治疗经验。方法:根据临床表现、X线、皮疹印片、皮疹和淋巴结活检,分析26例LCH的临床资料。结果:勒雪氏病(LS)18例,韩雪柯氏综合征(HSC)4例,骨嗜酸细胞肉芽肿(EGB)4例。诊断后给予放、化疗。1例尚未治疗即死亡,1例治疗中病情加重而死亡,6例放弃治疗失访,3例治疗不久尚未好转即放弃失访,9例治疗好转出院后失访,6例完成疗程无复发。结论:重视临床表现、特征性皮疹和皮疹印片,充分认识典型X线片有助于早期诊断。LS、HSC和多发骨损害的EGB联合化疗,单发EGB手术或放疗可改善预后。长春新碱(V),足叶乙甙(E),强的松(P)联合化疗效果好,不良反应轻。

Object:To summarize the experience of diagnosis and treatment of langerhans cell histiocytosis(LCH).Methods:To analyse the clinical materials of26cases with LCH.The diagnostic methods were based on clinical manifestations,radiography,rash print slice,rash biopsy,lymph node biopsy.Results:Letterer-siwee(LS)18cases,Hand-Sch ller-Christian(HSC)4cases,Eosinophilic granuloma of bone(EGB)4cases.After diagnosis the patients were treated with chemotherapy and radiotherapy.One case died before therapy.The condition wors-ened in one case and died during treatment.Six cases gave up treatment and failed to fallow.Three cases gave up treatment soon because the condition didn't improve after therapy began and failed to follow.Nine cases dischaged from hospital after improvement and failed to follow.Six cases completed the treatment and didn't relapse.Conclusion:Pay great attention to the protean clinical manifestation,the characteristics of the rash and rash print,full understanding the typical radiogram are helpful in early diagnosis.Combined chemotherapy for LS,HSC and multifocal bone lesions,surgical operation or radiotherapy for unifocal EGB may improve the prognosis.Combined chemotherapy with vincristine(V),etoposide(E),predisone(P)were superior to other chemothera-py in this series and the adverse reactions were slight.