Annino L, Ferrari A, Cedrone M, Giona F, Lo Coco F, Meloni G, Arcese W, Mandelli F
Department of Human Biopathology, University La Sapienza, Rome, Italy.
Leukemia. 1994 Apr;8(4):664-7.
Adult Philadelphia-chromosome-positive acute lymphoblastic leukemia (Ph1-positive ALL) represents about 30% of all adult ALL, and is considered a poor prognosis disease, since complete remission (CR), which can be achieved in 50-70% of cases, is usually short in patients treated with conventional chemotherapy. Presently bone marrow transplantation, performed early in first CR is becoming the treatment of choice, as it has shown to be able to cure some cases. In a ten-year period, at our department, among 108 adult ALL patients, in which cytogenetics was successfully carried out at diagnosis, 24 (22%) resulted Ph1-positive. Molecular biology was performed in 13 out of these 24 patients: 10 patients showed a p210 rearrangement and three p190. All patients have been treated at induction with conventional drugs, with a CR rate of 96%. As post-remission therapy, the first 17 cases (group 1) followed a chemotherapeutic program, like the other adult ALL; while the remaining six patients (group 2) have been enrolled in a pilot study including early BM transplantation. In group 1, the median overall duration of first CR is 7 months; 50% of relapses were recorded within the first 6 months, although in this group five patients exhibited a first CR prolonged more than 30 months. In group 2, among the six patients, three were submitted to allogeneic bone marrow transplantation (BMT), and three to autologous bone marrow transplantation (ABMT). Overall median duration of first CR is 13 months. Three patients relapsed, three are in continuous CR for 11, 31 and 32 months.
成人费城染色体阳性急性淋巴细胞白血病(Ph1阳性ALL)约占所有成人ALL的30%,被认为是一种预后不良的疾病,因为在50 - 70%的病例中可实现的完全缓解(CR),接受传统化疗的患者缓解期通常较短。目前,在首次完全缓解早期进行的骨髓移植正成为首选治疗方法,因为已证明它能够治愈一些病例。在十年期间,在我们科室,108例成人ALL患者中,诊断时成功进行了细胞遗传学检查,其中24例(22%)为Ph1阳性。这24例患者中有13例进行了分子生物学检查:10例患者显示p210重排,3例显示p190重排。所有患者诱导期均接受传统药物治疗,CR率为96%。作为缓解后治疗,前17例患者(第1组)遵循化疗方案,与其他成人ALL患者一样;而其余6例患者(第2组)参加了一项包括早期骨髓移植的试点研究。在第1组中,首次CR的中位总持续时间为7个月;50%的复发发生在头6个月内,尽管该组有5例患者首次CR持续时间超过30个月。在第2组的6例患者中,3例接受了异基因骨髓移植(BMT),3例接受了自体骨髓移植(ABMT)。首次CR的总体中位持续时间为13个月。3例患者复发,3例持续CR 11、31和32个月。