Giona F, Testi A M, Rondelli R, Amadori S, Arcese W, Meloni G, Moleti M L, Ceci A, Pillon M, Madon E, Comis M, Pession A, Mandelli F
Ematologia, Dipartimento di Biotecnologie Cellulari ed Ematologia, Università La Sapienza, Roma, Italy.
Br J Haematol. 1997 Dec;99(3):671-7. doi: 10.1046/j.1365-2141.1997.4413253.x.
Seventy-three children with acute lymphoblastic leukaemia (ALL) in first bone marrow (BM) relapse, occurring within 30 months from complete remission (CR), were enrolled in an Italian cooperative study (ALL R-87 protocol). This treatment programme consisted of an induction phase with intermediate-dose cytarabine (IDARA-C) plus idarubicin (IDA) and prednisone (PDN), followed by a multidrug consolidation therapy and bone marrow transplant (BMT). 55/73 children achieved CR (75.3%); 15 (20.5%) failed to respond and three (4.2%) died during induction. The response rate was significantly higher for children with a first CR duration > or = 12 months (P=0.0005) and for those with a white blood cell (WBC) count at relapse < 20 x 10(9)/l (P=0.004). The estimated disease-free survival (DFS +/- SE) at 82 months was 0.18 +/- 0.05 for all responders, and 0.70 +/- 0.14 for allotransplanted patients versus 0.05 +/- 0.05 for those autografted (P=0.001). The estimated probabilities of survival +/- SE and event-free survival (EFS +/- SE) at 83 months were 0.16 +/- 0.07 and 0.13 +/- 0.04, respectively. for all enrolled children. Univariate analysis showed that age < 10 years at initial diagnosis and B-lineage immunophenotype favourably influenced both DFS (P=0.001) and EFS probabilities (P=0.0014 and P=0.012, respectively), whereas a first CR duration > or = 12 months and a WBC count at relapse < 20 x 10(9)/l were associated only with a better EFS rate (P=0.026 and P=0.004, respectively). Our results show the efficacy of the IDA plus IDARA-C schedule used in the ALL R-87 protocol in high-risk relapsed ALL children. Allogeneic BMT proved effective for patients with an HLA sibling donor. In a multivariate analysis, age > or = 10 years at initial diagnosis (P=0.016) and WBC count at relapse > or = 20 x 10(9)/l (P=0.048) were independently associated with a worse disease outcome.
73名急性淋巴细胞白血病（ALL）首次骨髓（BM）复发的儿童，在完全缓解（CR）后30个月内复发，参加了一项意大利合作研究（ALL R - 87方案）。该治疗方案包括一个诱导期，使用中剂量阿糖胞苷（IDARA - C）加伊达比星（IDA）和泼尼松（PDN），随后进行多药巩固治疗和骨髓移植（BMT）。73名儿童中有55名实现了CR（75.3%）；15名（20.5%）无反应，3名（4.2%）在诱导期死亡。首次CR持续时间≥12个月的儿童（P = 0.0005）以及复发时白细胞（WBC）计数<20×10⁹/L的儿童（P = 0.004）的缓解率显著更高。所有缓解者在82个月时的估计无病生存率（DFS±SE）为0.18±0.05，接受同种异体移植的患者为0.70±0.14，而自体移植患者为0.05±0.05（P = 0.001）。所有入组儿童在83个月时的估计生存概率±SE和无事件生存率（EFS±SE）分别为0.16±0.07和0.13±0.04。单因素分析显示，初始诊断时年龄<10岁和B系免疫表型对DFS（P = 0.001）和EFS概率（分别为P = 0.0014和P = 0.012）有有利影响，而首次CR持续时间≥12个月和复发时WBC计数<20×10⁹/L仅与更好的EFS率相关（分别为P = 0.026和P = 0.004）。我们的结果显示了ALL R - 87方案中使用的IDA加IDARA - C方案对高危复发ALL儿童的疗效。同种异体BMT对有HLA同胞供体的患者证明有效。在多因素分析中，初始诊断时年龄≥10岁（P = 0.016）和复发时WBC计数≥20×10⁹/L（P = 0.048）与更差的疾病结局独立相关。
