用依托泊苷和氮丙啶基苯醌治疗淋巴细胞组织细胞性红细胞吞噬作用。

Treatment of lymphohistiocytic erythrophagocytosis with VP-16 and aziridinylbenzoquinone.

作者信息

Becton D L, Kurtzberg J, Kinney T R, Friedman H S, Chaffee S, Falletta J M

出版信息

Med Pediatr Oncol. 1987;15(2):58-61. doi: 10.1002/mpo.2950150203.

Abstract

Lymphohistiocytic erythrophagocytosis (LE) is a usually fatal disease characterized by fever, organomegaly, hyperlipidemia, central nervous system involvement, and cellular immunodeficiency. Treatment with corticosteroids, cytotoxic chemotherapy, and blood exchange is unsuccessful. We have treated two children with the epipodophyllotoxin VP-16 and with intrathecal chemotherapy. Each patient had an initial complete response, and one remains in remission 36 months after therapy began. Aziridinylbenzoquinone (AZQ) therapy induced a complete response in a patient who relapsed during VP-16 therapy. A combination of VP-16 and intrathecal chemotherapy appears to be the most effective therapy for LE, and further evaluation of the role of AZQ is indicated.

摘要

淋巴细胞组织细胞性红细胞吞噬作用（LE）是一种通常致命的疾病，其特征为发热、器官肿大、高脂血症、中枢神经系统受累及细胞免疫缺陷。使用皮质类固醇、细胞毒性化疗和血液置换治疗均未成功。我们用表鬼臼毒素VP - 16和鞘内化疗治疗了两名儿童。每名患者最初均有完全缓解，其中一名在治疗开始36个月后仍处于缓解状态。氮丙啶基苯醌（AZQ）治疗使一名在VP - 16治疗期间复发的患者获得完全缓解。VP - 16和鞘内化疗联合似乎是治疗LE最有效的方法，有必要进一步评估AZQ的作用。