Miniero R, Saracco P, Pastore G, Zurlo M G, Terracini B, Rosso P, Masera G
Department of Pediatrics, University of Turin, Italy.
Med Pediatr Oncol. 1995 Feb;24(2):71-6. doi: 10.1002/mpo.2950240202.
The outcome of 171 children with ALL who relapsed for the first time after elective cessation of therapy (1-86 mo) and followed over 10 years (median 60 mo; range 1-232 mo) has been evaluated. One hundred and three patients relapsed in the bone marrow (BM), 29 in the testis (T), 21 in the central nervous system (CNS), 14 in the BM plus another site and 4 in other sites. Second remission was achieved in 97% of patients (97% BM, 100% T, 90% CNS, respectively) with reinduction schedules including three or more drugs. All but 4 out of 100 patients who relapsed in the BM received cranial reprophylaxis with intrathecal CT alone or CT plus radiotherapy. Seven patients in second CR underwent allogeneic bone marrow transplantation from an HLA matched sibling. The overall survival was 34% and disease-free survival (DFS) probability at 100 years was 22%. A second relapse was observed in 73% of patients. Forty children are alive in second continuous remission and 24 are alive after a second or subsequent relapse. Patients with isolated T relapse showed a significant better outcome than those with BM or CNS involvement. Most patients (62%) with isolated BM relapse showed a further disease recurrence in BM, and DFS was shorter when relapse occurred within 12 months from off-therapy. Eighty-two patients in second CR stopped the treatment a second time and showed a survival and DFS probabilities, respectively, of 69% and 43%. Thus, children with ALL who relapse after cessation of therapy still have a high risk of further late relapses and should be treated with intensive chemotherapy and CNS reprophylaxis. BMT must be considered for all patients relapsing in the BM within 12 months from off-therapy.
对171例急性淋巴细胞白血病（ALL）患儿进行了评估，这些患儿在选择性停止治疗（1 - 86个月）后首次复发，并随访了10年（中位时间60个月；范围1 - 232个月）。103例患者骨髓（BM）复发，29例睾丸（T）复发，21例中枢神经系统（CNS）复发，14例骨髓合并其他部位复发，4例其他部位复发。97%的患者获得第二次缓解（骨髓复发患者中97%、睾丸复发患者中100%、中枢神经系统复发患者中90%），再诱导方案包括三种或更多药物。100例骨髓复发患者中除4例之外均接受了单纯鞘内化疗（CT）或CT联合放疗的颅脑预防性治疗。第二次完全缓解（CR）的7例患者接受了来自HLA匹配同胞的异基因骨髓移植。总生存率为34%，100年无病生存率（DFS）概率为22%。73%的患者出现第二次复发。40例患儿处于第二次持续缓解状态存活，24例在第二次或后续复发后存活。单纯睾丸复发的患者预后明显好于骨髓或中枢神经系统受累的患者。大多数（62%）单纯骨髓复发的患者骨髓再次复发，且从停止治疗起12个月内复发时DFS较短。第二次CR的82例患者再次停止治疗，其生存率和DFS概率分别为69%和43%。因此，停止治疗后复发的ALL患儿仍有很高的进一步晚期复发风险，应接受强化化疗和中枢神经系统预防性治疗。对于停止治疗后12个月内骨髓复发的所有患者都必须考虑进行骨髓移植。
