Ostronoff M, Soussain C, Zambon E, Ibrahim A, Bosq J, Bayle C, Pico J L, Gilles E, Moran A, Droz J P
Service d'Hématologie, Institut Gustave Roussy, Villejuif, France.
Nouv Rev Fr Hematol (1978). 1992;34(5):389-97.
The prognosis of Burkitt's lymphoma is generally considered to be poor, particularly in the advanced stages of the disease. Although recent chemotherapy protocols have given high rates of cure in children, there are few such reports concerning adults. We therefore conducted a retrospective analysis of the results for treatment of 46 adults in the Institut Gustave Roussy (IGR) between 1978 and 1987, in order to establish an effective treatment strategy for use in a prospective trial. The median age of the patients was 31 years and the majority were Caucasians of European origin. The clinical symptoms and course of the disease were similar to those in the pediatric situation and corresponded to the so-called non endemic forms observed in Europe and the United States. Presentation was generally extra-nodal, usually with abdominal manifestations. As a rule, the disease progressed rapidly and showed a high affinity for the central nervous system in the absence of specific prophylaxis, despite systemic therapy with highly active agents, particularly in the advanced stages. According to Murphy's classification, there were 6 stage I, 11 stage II, 19 stage III and 10 stage IV patients (3 of whom had CNS involvement). Treatment was heterogenous, although all the patients received polychemotherapy including anthracyclins. The best results were obtained from eleven patients treated according to the French Multicenter Protocols for pediatric Burkitt's lymphoma (LMB-84 and LMB-86). Kaplan-Meier 5-year relapse free survival rate among the 46 patients was 42% (stage I: 83%; stage II: 67%; stage III: 30%; stage IV: 30%). In order to standardize our therapeutic approach, we started a prospective study in 1988 using the unmodified pediatric protocol for our adult patients.
伯基特淋巴瘤的预后通常被认为较差，尤其是在疾病的晚期阶段。尽管最近的化疗方案在儿童中取得了较高的治愈率，但关于成人的此类报道较少。因此，我们对1978年至1987年间在古斯塔夫·鲁西研究所（IGR）接受治疗的46名成人患者的结果进行了回顾性分析，以便制定一种有效的治疗策略用于前瞻性试验。患者的中位年龄为31岁，大多数是欧洲血统的白种人。该疾病的临床症状和病程与儿童情况相似，与在欧洲和美国观察到的所谓非地方性形式相符。通常表现为结外病变，常见腹部表现。一般来说，疾病进展迅速，在没有特异性预防措施的情况下，即使使用高效药物进行全身治疗，尤其是在晚期阶段，对中枢神经系统也有很高的亲和力。根据墨菲分类法，有6例I期、11例II期、19例III期和10例IV期患者（其中3例有中枢神经系统受累）。治疗方法各不相同，尽管所有患者都接受了包括蒽环类药物在内的多药化疗。根据法国儿童伯基特淋巴瘤多中心方案（LMB - 84和LMB - 86）治疗的11名患者取得了最佳结果。46例患者的Kaplan - Meier 5年无复发生存率为42%（I期：83%；II期：67%；III期：30%；IV期：30%）。为了规范我们的治疗方法，我们于1988年开始了一项前瞻性研究，对成年患者使用未修改的儿童方案。
