Service de Pédiatrie, Hôpital Gabriel Touré, Bamako, Mali.
Pediatr Blood Cancer. 2011 Jan;56(1):70-6. doi: 10.1002/pbc.22746.
The French African Group of Pediatric Oncology was set-up to improve quality of care for children with cancer. Preliminary observations on the efficacy in Burkitt lymphoma (BL) of a cyclophosphamide monotherapy (CPM) have been published. We report the results of a multicentric prospective study combining first-line CPM and a multidrug second-line chemotherapy (SC) for refractory/relapsed patients.
Patients ≤ 18 years with Burkitt or Burkitt-like lymphoma, were included in six countries (Burkina-Faso, Cameroon, Ivory Coast, Madagascar, Mali, and Senegal). All patients received three weekly CPM courses (1.2 g/m(2) IV with intrathecal methotrexate and hydrocortisone), stage 3/4 patients received three further courses. SC added methotrexate, vincristine, cytarabine, and prednisone.
There were 178 patients included (42 stage 1/2, 134 stage 3/4, and 2 unknown). Isolated facial localization was found in 41 patients, diffuse abdominal involvement in 120 patients including 65 with both. Nine early deaths were reported, toxicity occurred in 136/743 courses (83 patients) and was predominantly hematological. After CPM, complete remission (CR) rate was 47% with a 33% EFS. Because of rapid progression 76/108 eligible patients (85 primary refractory and 23 relapses) received SC resulting in 35.7% CR but a 21% toxic death rate. The OS of the whole strategy was 50.5% and correlated to stage.
A prospective multicentric study on BL was feasible in very low-income countries. CPM can be recommended in stage 1-2 because of optimal cost/benefit ratio. However, more intensive strategies, still adapted to socio-economic conditions, are required for advanced stages 3 and 4.
法国非洲儿科肿瘤学组织成立的目的是提高癌症患儿的治疗质量。此前已经发表了环磷酰胺单药治疗（CPM）治疗伯基特淋巴瘤（BL）的初步疗效观察结果。我们报告了一项多中心前瞻性研究的结果，该研究将一线 CPM 与多药二线化疗（SC）联合用于难治/复发患者。
纳入了来自六个国家（布基纳法索、喀麦隆、科特迪瓦、马达加斯加、马里和塞内加尔）的≤18 岁的伯基特或伯基特样淋巴瘤患者。所有患者均接受了三个每周一次的 CPM 疗程（1.2 g/m2 静脉注射，同时鞘内给予甲氨蝶呤和氢化可的松），III/IV 期患者还接受了三个额外的疗程。SC 添加了甲氨蝶呤、长春新碱、阿糖胞苷和泼尼松。
共纳入了 178 例患者（42 例为 I/II 期，134 例为 III/IV 期，2 例不详）。41 例患者为孤立性面部定位，120 例患者为弥漫性腹部受累，其中 65 例患者同时存在这两种情况。报告了 9 例早期死亡，136/743 个疗程（83 例患者）发生了毒性，主要为血液学毒性。CPM 后，完全缓解（CR）率为 47%，无事件生存率（EFS）为 33%。由于病情快速进展，76/108 例符合条件的患者（85 例初治耐药和 23 例复发）接受了 SC 治疗，CR 率为 35.7%，但毒性死亡率为 21%。整个策略的总生存率（OS）为 50.5%，与分期相关。
在非常低收入国家进行 BL 的前瞻性多中心研究是可行的。由于成本效益比最佳，CPM 可推荐用于 I/II 期。然而，对于 III/IV 期，需要更强化的策略，仍然需要适应社会经济条件。
