Department of Paediatric Haemato-Oncology, Royal Marsden Hospital NHS Foundation Trust, Sutton, Surrey, UK.
Leuk Lymphoma. 2012 Oct;53(10):1882-8. doi: 10.3109/10428194.2012.677534. Epub 2012 Apr 23.
Patients with childhood relapsed and refractory mature B-cell non-Hodgkin lymphoma (B-NHL) and acute lymphoblastic leukemia (B-ALL) are rare and have a dismal prognosis. The previous UK national analysis of 26 children over a 7-year period prior to 1996 had highlighted the poor outcome, with only three survivors. This 10-year multicenter study evaluated recent data, since 2000. Of 33 children, nine survived (27.3%), with a median follow-up of 4.3 years. On exclusion of six children treated with palliative intent, the survival was one-third (nine of 27; 33.3%). All patients with primary refractory disease (n = 7) and all except one with early relapse (n = 11) died. Administration of four doses of 375 mg/m(2) of rituximab was associated with a longer survival (p = 0.006). Response to reinduction (p < 0.001) and autologous hematopoietic stem cell transplant (auto-HSCT) (p = 0.003) were significant on multivariate analysis. Patients with a time to relapse of at least 6 months are potentially curable and must be offered intensive treatment with salvage chemotherapy, rituximab and auto-HSCT.
患有儿童复发性和难治性成熟 B 细胞非霍奇金淋巴瘤 (B-NHL) 和急性淋巴细胞白血病 (B-ALL) 的患者较为罕见，且预后较差。在 1996 年之前的 7 年时间里，英国曾对 26 名儿童进行了全国性分析，结果显示预后不良，仅有 3 名幸存者。这项为期 10 年的多中心研究评估了自 2000 年以来的最新数据。在 33 名儿童中，有 9 人存活（27.3%），中位随访时间为 4.3 年。排除 6 名接受姑息治疗的儿童后，存活率为三分之一（27 名中的 9 名；33.3%）。所有原发性难治性疾病患者（n=7）和除 1 名早期复发患者（n=11）均死亡。接受 4 剂 375mg/m2 的利妥昔单抗治疗与更长的生存时间相关（p=0.006）。多变量分析显示，再次诱导缓解（p<0.001）和自体造血干细胞移植（auto-HSCT）（p=0.003）与预后相关。复发时间至少为 6 个月的患者具有潜在的可治愈性，必须给予强化治疗，包括挽救性化疗、利妥昔单抗和 auto-HSCT。
