Lerede T, Bassan R, Rossi A, Di Bona E, Rossi G, Pogliani E M, Motta T, Torri V, Buelli M, Comotti B, Viero P, Rambaldi A, Cortelazzo S, Rodeghiero F, Barbui T
Department of Ospedali Riuniti di Bergamo, Italy.
Haematologica. 1996 Sep-Oct;81(5):442-9.
Adult B/L3-ALL is a rare disease subset characterized by an aggressive clinical course and a poor response to conventional adult ALL-type chemotherapy. Recent data from the GMALL Group showed that prognosis can be improved with an innovative treatment regimen. In the current retrospective survey we focus on therapeutic results obtained at our Institutions during a 15-year period with ALL-type regimens in 34 adults with either B/L3-ALL or advanced-stage Burkitt's lymphoma.
Five successive ALL treatment programs were developed. They included a homogeneous induction phase with early intrathecal chemoprophylaxis, multidrug postremission consolidation followed by cranial irradiation (4 trials), high-dose chemotherapy plus autografting (2 trials), late consolidation (2 trials), and variable-length maintenance (4 trials). Early response and prolonged disease-free survival rates were analyzed according to selected clinical and therapeutic variables.
Overall, a complete remission was achieved in 62%, with a median duration of 1.6 years and a 10-year remission rate of 49%. A diagnosis of B/L3-ALL (p = 0.007), the use of idarubicin instead of adriamycin during induction (p = 0.018), a serum creatinine < 1.6 mg/dL, and an uninvolved central nervous system were associated with higher response rates. As regards long-term disease-free survival, results were significantly better in patients with < 1 x 10(9)/L L3/blast cells in the peripheral blood (p = 0.0029) and/or aged < 50 years (p = 0.04), and in those consolidated with the most recent rotational high-dose plus peripheral blood stem cell autotransplant regimen.
According to the results presented, ALL-like regimens may still represent a worthwhile therapeutic choice. The use of idarubicin during induction, the prognostic subclassification of patients, a careful control of dysmetabolic complications, the selection of the proper chemo-radioprevention for meningeal disease and perhaps the introduction of high-dose chemotherapy supported by autologous stem cell rescue appear to be the mainstay of further improvements.
成人B/L3-急性淋巴细胞白血病（ALL）是一种罕见的疾病亚型，其临床病程侵袭性强，对传统成人ALL型化疗反应不佳。GMALL研究组的最新数据显示，采用创新治疗方案可改善预后。在本次回顾性研究中，我们关注了15年间在我们机构采用ALL型方案治疗的34例B/L3-ALL或晚期伯基特淋巴瘤成人患者的治疗结果。
制定了五个连续的ALL治疗方案。包括一个均一的诱导期，早期鞘内化疗预防，多药缓解后巩固治疗，随后进行颅脑照射（4项试验），高剂量化疗加自体移植（2项试验），晚期巩固治疗（2项试验），以及不同时长的维持治疗（4项试验）。根据选定的临床和治疗变量分析早期反应和延长的无病生存率。
总体而言，62%的患者实现了完全缓解，中位缓解持续时间为1.6年，10年缓解率为49%。B/L3-ALL的诊断（p = 0.007）、诱导期使用伊达比星而非阿霉素（p = 0.018）、血清肌酐<1.6 mg/dL以及中枢神经系统未受累与较高的缓解率相关。关于长期无病生存，外周血L3/原始细胞<1×10⁹/L（p = 0.0029）和/或年龄<50岁（p = 0.04）的患者，以及采用最新的旋转高剂量加外周血干细胞自体移植方案巩固治疗的患者，结果明显更好。
根据所呈现的结果，ALL样方案可能仍然是一种值得的治疗选择。诱导期使用伊达比星、患者的预后亚分类、对代谢紊乱并发症的仔细控制、为脑膜疾病选择合适的化疗放疗预防措施，以及或许引入自体干细胞救援支持的高剂量化疗似乎是进一步改善的关键。
