Vowels M R, Lam-Po-Tang R, Mameghan H, Heller E, Ford D, Ziegler J, Hughes D O
Cancer. 1985 Dec 15;56(12):2786-8. doi: 10.1002/1097-0142(19851215)56:12<2786::aid-cncr2820561212>3.0.co;2-e.
This report describes a girl who was diagnosed with malignant histiocytosis at the age of 5 years. The disease was controlled initially with chemotherapy for 3 years and had then recurred with meningeal involvement on three occasions. Four years and 8 months from diagnosis, bone marrow transplantation (BMT) was undertaken from an HLA-identical and mixed lymphocyte culture (MLC) nonreactive brother after conditioning with VP-16-213 5 mg/kg/day X 2, cyclophosphamide 60 mg/kg/day X 2, and total body irradiation 200 rad twice daily to a total dose of 1000 rad delivered at 7 rad/minute. At the time of transplant, the disease was in remission. Currently, more than 48 months after the transplant, the child remains free of disease, with a normally functioning donor marrow and with no evidence of graft versus host disease. This is the first recorded case of BMT in the treatment of malignant histiocytosis. The outcome in this patient in late-stage disease suggests that BMT could be considered early in management as definitive therapy.
本报告描述了一名5岁时被诊断为恶性组织细胞增多症的女孩。该病最初通过化疗控制了3年,随后三次复发并累及脑膜。确诊后4年零8个月,在接受以下预处理后,由一名HLA相同且混合淋巴细胞培养(MLC)无反应的哥哥进行了骨髓移植(BMT):依托泊苷(VP - 16 - 213)5毫克/千克/天,共2天;环磷酰胺60毫克/千克/天,共2天;全身照射,每天2次,每次200拉德,以7拉德/分钟的速度给予,总剂量为1000拉德。移植时,疾病处于缓解期。目前,移植后已超过48个月,该患儿仍无疾病迹象,供体骨髓功能正常,且无移植物抗宿主病的证据。这是首例记录在案的采用骨髓移植治疗恶性组织细胞增多症的病例。该晚期疾病患者的治疗结果表明,在治疗初期可考虑将骨髓移植作为确定性治疗方法。