应用改良LCH-Ⅰ方案治疗郎格罕细胞组织细胞增生症疗效观察

摘要

探讨国际组织细胞协会制定的LCH-Ⅰ方案的治疗效果，提高重症郎格罕细胞组织细胞增生症(LCH)患儿的疗效。方法17例LCH均经临床、实验室检查及活组织病理检查确诊。按hvin-Osband评分方法将疾病严重程度分为4级。Ⅰ～Ⅳ级均采用改良LCH-Ⅰ方案，即先予大剂量甲基泼尼松龙冲击治疗，继以8 个疗程的鬼臼噻酚甙(VM26)完成整个治疗。按协会疗效标准进行评价。结果Lavin-Osband分级：Ⅰ级8例、 Ⅱ级1例、Ⅲ级2例、Ⅳ级6例。短期疗效Ⅲ、Ⅳ级8例全部好转，有效率为100％；Ⅰ、Ⅱ级6例好转，有效率为 66.7％。追踪结果：Ⅲ、Ⅳ级2例治愈、5例好转、1例恶化；Ⅰ、Ⅱ级6例好转，3例稳定，总有效率为76.5％ (13/17)。结论新的诊断标准明确，与国际标准接轨。LCH-Ⅰ方案疗效显著，尤对治疗时年龄＜1岁、病程＜ 3个月、病情重及S-100蛋白阳性者更显其优越性。%Objective To explore the treatment results by using modifiedLCH-Ⅰ protocol which was made by International Study Group of LCH. Try to improve the treatment result of severe patients. Methods 17 cases were diagnosed as LCH according to clinical manifestation,laboratory tests and bone or skin lesions biopsies. All the cases were scored according to Lavin-Osband criteria. Treatment protocol included: one course of high-dose methylprednisolone followed by 8 courses of teniboside ( VM26 ). The efficacy of the treatment was evaluated according to the efficacy criteria of International Study Group of the Histocyte Society. Results The Lavin-Osband classification showed:8 cases were type Ⅰ, 1 cases was Ⅱ,2 cases were Ⅲ and 6 cases were Ⅳ. The treatment results showed obvious clinical improvement in 8 cases for type Ⅲ～Ⅳ after treatment. The effective rate was 100%. For type Ⅰ～Ⅱ,only 6 cases got obvious improvement after treatment. The effective rate was 66. 7%. The long-time follow-up results showed: in type Ⅲ～Ⅳ, 2 cases recovered completely,5 cases greatly improved,1 case deteriorated;in type Ⅰ～Ⅱ,6 cases greatly improved,3 cases stable. The total effective rate was 76. 5%. Conclusions The new standard of diagnosis and classification are clear. It is close to the international standard. The better treatment result belongs to those patients who are younger than 1 year old,months of history shorter than 3,higher score and the S-100 protein positive.