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背景与目的:儿童青少年淋巴母细胞型淋巴瘤属于高度恶性淋巴瘤,进展快,死亡率高。德国BFM-90淋巴母方案是目前治疗儿童青少年淋巴母细胞型淋巴瘤疗效最好的方案之一。本研究采用改良BFM-90淋巴母方案治疗中国儿童青少年淋巴母细胞型淋巴瘤,观察其疗效、毒性和可行性。方法:20例3～18岁初治的T淋巴母细胞淋巴瘤患者,Ⅲ期7例,Ⅳ期13例。18例(90%)患者有纵隔肿块伴上腔静脉阻塞综合征,10例(50%)有骨髓侵犯。所有患者均接受改良BFM-90方案化疗,方案包括诱导缓解、巩固治疗和中枢神经系统预防、再诱导缓解和维持治疗。总疗程2年。用Kaplan-Meier法统计全组生存率。结果:诱导缓解结束后18例(90%)患者获得完全缓解(completeresponse,CR),1例(5%)部分缓解(partialresponse,PR),1例(5%)肿瘤进展(progressivedisease,PD),总有效率95%。PR和PD的2例患者最后肿瘤进展死亡。CR的18例患者中有2例在再诱导结束后CR1时行外周血自体造血干细胞移植。移植后有1例复发,经再次化疗后CR存活;另1例一直存活。其他CR的患者中有5例复发,其中1例行异基因移植后存活,1例自体造血干细胞移植后存活,3例复发后单纯化疗的患者肿瘤进展死亡。全组患者3年总生存率74%。所有患者在诱导和再诱导阶段均发生Ⅲ/Ⅳ骨髓抑制,积极对症处理后可恢
BACKGROUND & OBJECTIVE: Child and adolescent lymphoblastic lymphoma is a highly malignant lymphoma with rapid progression and high mortality. Germany BFM-90 lymphoblast program is currently the best treatment of children and adolescents lymphoblastic lymphoma one of the programs. This study used modified BFM-90 lymphoblastoid regimen to treat children and adolescent lymphoblastic lymphoma in China and observed its curative effect, toxicity and feasibility. Methods Twenty patients with T lymphoblastic lymphoma who were 3 to 18 years old, including 7 cases in stage Ⅲ and 13 cases in stage Ⅳ, were included in this study. Eighteen patients (90%) had mediastinum mass with superior vena cava obstruction syndrome and 10 patients (50%) had bone marrow invasion. All patients underwent modified BFM-90 regimen, including induction of remission, consolidation and central nervous system prophylaxis, re-induction of remission and maintenance of treatment. The total course of treatment for 2 years. The overall survival was calculated using the Kaplan-Meier method. RESULTS: Eighteen patients (90%) had complete response (CR), partial response (PR) in 1 case (5%), progressived disease (PD) in 1 case (5% The total efficiency of 95%. Two patients with PR and PD eventually died of tumor progression. Two of the 18 CR patients underwent peripheral blood autologous stem cell transplantation at CR1 after re-induction. One case had recurrence after transplantation and survived after another chemotherapy. Another case survived. Five of the other CR patients relapsed, of which one survived after allogeneic transplantation, one survived after autologous stem cell transplantation, and three patients who had recurred after chemotherapy had died of tumor progression. The overall 3-year overall survival rate of 74%. All patients had stage III / IV myelosuppression during induction and re-induction phase, and recovered after active symptomatic treatment