Pediatric oncology group experience with modified LSA2‐L2 therapy in 107 children with non‐Hodgkin's lymphoma (Burkitt's lymphoma excluded)
Pediatric oncology group experience with modified LSA2‐L2 therapy in 107 children with non‐Hodgkin's lymphoma (Burkitt's lymphoma excluded)
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小儿肿瘤小组对 107 名非霍奇金淋巴瘤儿童（伯基特淋巴瘤除外）采用改良 LSA2-L2 疗法的经验
作者：
M. Sullivan;J. Boyett;J. Pullen;W. Crist;E. Doering;R. Trueworthy;E. Hvizdala;F. Ruymann;C. Steuber
从 1976 年 9 月到 1979 年 8 月，儿科肿瘤学小组访问了 145 名儿童，研究改良 LSA2-L2 疗法治疗非霍奇金淋巴瘤 (NHL) 的有效性。伯基特淋巴瘤患者不符合资格； 1977 年 2 月之后进入的骨髓中原始细胞≥25% 的 E-玫瑰花结阳性患者单独报告。放射治疗可用于治疗诊断时患有压迫性纵隔疾病的患者，并且适用于诱导化疗完成后进行二次手术证明有残留腹部疾病的患者。病理学小组和淋巴瘤临床试验储存中心必须确认诊断。对 131 名患者的诊断组织进行了审查。在 107 名可评估患者中，91 名（85%）获得完全缓解。三个主要组织学组（淋巴母细胞、未分化和大细胞）之间的缓解率差异具有统计学意义，其中弥漫性未分化淋巴瘤的缓解最差（P = 0.03）。三种主要组织学诊断的无失败生存率没有显着差异。虽然墨菲 III 期患者的缓解率最低 (79%)，但各阶段之间的差异并不显着。分期并不是无失败生存的重要预后因素（P = 0.08）。仍处于危险中的患者人数以及 Kaplan-Meier 对 3 年后仍处于危险中的患者百分比的估计为： I 期，8（100%）；第二阶段，10（67%）；第三阶段，28（57%）；第四阶段，6（39%）；和 > 25% 母细胞，1 (13%)。淋巴母细胞疾病的 III 期失败曲线显示 3 年内持续逐步失败。在患有弥漫性大细胞和未分化疾病的患者中，大多数失败发生在 8 个月时。骨髓受累的 M1 和 M2 水平不能预测患有淋巴母细胞疾病的儿童。纵隔肿块的存在是导致无骨髓受累的淋巴母细胞疾病儿童失败的一个重要因素。白细胞增多> 10,000/1，是预测大细胞淋巴瘤患者无失败生存的重要因素（P = <0.001）。放射治疗并不是实现缓解的重要因素。二次探查显示，使用放射疗法治疗诱导后残留病并没有产生一致的益处。 LSA2-L2 方案与相当大的毒性相关，77% 的患者出现严重或更严重的毒性，其中 40% 的患者危及生命。四人死于中毒。然而，随着研究者经验的增加，治疗变得更加容易和安全。在大多数情况下，维持治疗是在门诊进行的，没有出现并发症。这项研究证明了 LSA2-L2 方案能够产生长期缓解，或“治愈”儿童 NHL 主要组织学类别中 60% 至 70% 的儿童（伯基特淋巴瘤除外）。 90% 的 I 期和 II 期疾病患者实现长期生存或“治愈”，约 50% 的 III 期和 IV 期疾病患者实现长期生存。放疗和二次手术似乎都不影响结果。累及纵隔的淋巴母细胞疾病的持续复发模式表明需要“预先”进行更强化的重复治疗。非淋巴母细胞疾病的复发模式表明一年后停止治疗。
From September 1976 to August 1979 the Pediatric Oncology Group accessed 145 children to study the effectiveness of modified LSA2‐L2 therapy for the treatment of non‐Hodgkin's lymphoma (NHL). Burkitt's lymphoma patients were ineligible; E‐rosette‐positive patients with ≥ 25% blasts in the marrow entered after February 1977 were reported separately. Radiotherapy could be used to treat patients with compressive mediastinal disease at diagnosis and was prescribed for those with residual abdominal disease as demonstrated by second‐look surgery on completion of induction chemotherapy. Confirmation of diagnosis by the Pathology Panel and Repository Center for Lymphoma Clinical Trials was mandatory. Diagnostic tissues of 131 patients were reviewed. Among 107 evaluable patients, 91 (85%) achieved complete remission. Differences in response rates among the three major histologic groups (lymphoblastic, undifferentiated, and large cell) were of statistical significance, with response being poorest for diffuse undifferentiated lymphoma (P = 0.03). Failure‐free survival did not differ significantly for the three major histologic diagnoses. While response rate was lowest for Murphy Stage III patients (79%), the differences among the stages were not significant. Stage was not a significant prognostic factor for failure‐free survival (P = 0.08). The number of patients still at risk and the Kaplan‐Meier estimate of percentage of patients remaining at risk after 3 years is: Stage I, 8 (100%); Stage II, 10 (67%); Stage III, 28 (57%); Stage IV, 6 (39%); and > 25% blasts, 1 (13%). Stage III failure curves for lymphoblastic disease show continuing stepwise failure through 3 years. Among patients with diffuse large cell and undifferentiated disease, most failures occurred by 8 months. M1 and M2 levels of marrow involvement were not prognostic among children with lymphoblastic disease. The presence of a mediastinal mass was a significant factor contributing to failure in children with lymphoblastic disease without marrow involvement. Leucocytosis > 10,000/1, was a significant (P = <0.001) factor predicting failure‐free survival for patients with large cell lymphoma. The delivery of radiotherapy was not a significant factor in achieving remission. No consistent benefit resulted from using radiotherapy to treat postinduction residual disease demonstrated on second‐look exploration. The LSA2‐L2 regimen was associated with considerable toxicity, severe or worse in 77% and life‐threatening to 40% of these patients. Four died of toxicity. However, therapy was given more easily and safely as investigator experience increased. Maintenance therapy was delivered on an out patient basis without complications in most instances. This study demonstrates the ability of the LSA2‐L2 regimen to produce long‐term remissions, or to “cure” 60% to 70% of children among the major histologic categories of childhood NHL, excepting Burkitt's lymphoma. Long‐term survival, or “cure”, occurs in 90% with Stages I and II disease and some 50% of those with Stages III and IV disease. Neither radiotherapy nor second‐look surgical procedures appear to influence outcome. The continuous relapse pattern seen in lymphoblastic disease involving the mediastinum suggests the need for more intensive repetitive therapy “up‐front”. The relapse pattern of non‐lymphoblastic disease suggests the discontinuation of therapy after 1 year.
霍奇金病的手术重新分期。
DOI：
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发表时间：
1982
期刊：
Cancer treatment reports
影响因子：
--
作者：
Goodman,GE;Jones,SE;Villar,HV;Silverstein,ME;Dabich,L;Newcome,SR
通讯作者：
Newcome,SR
儿童 T 细胞淋巴恶性肿瘤表面抗原的单克隆抗体表征。
影响因子：
20.3
作者：
Roper,M;Crist,WM;Metzgar,R;Ragab,AH;Smith,S;Starling,K;Pullen,J;Leventhal,B;Bartolucci,AA;Cooper,MD
通讯作者：
Cooper,MD
53 名 E-rosette 阳性 T 细胞白血病儿童的改良 LSA2-L2 治疗：结果和预后因素（一项儿科肿瘤学小组研究）。
影响因子：
20.3
作者：
Pullen,DJ;Sullivan,MP;Falletta,JM;Boyett,JM;Humphrey,GB;Starling,KA;Land,VJ;Dyment,PG;Vats,T;Duncan,MH
通讯作者：
Duncan,MH