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Treatment of relapsed/refractory paediatric aggressive B-cell non-Hodgkin lymphoma.
British Journal of Haematology ( IF 6.5 ) Pub Date : 2020-03-06 , DOI: 10.1111/bjh.16461
Maria L Moleti 1 , Anna M Testi 1 , Robin Foà 1
Affiliation  

Aggressive B‐cell non‐Hodgkin lymphoma (B‐NHL) accounts for ≈60% of NHL in children/adolescents. In newly diagnosed Burkitt lymphoma and diffuse large B‐cell lymphoma, short intensive multiagent chemotherapy is associated with a five‐year event‐free survival of around 90%. Very few children/adolescents with aggressive B‐NHL show a relapsed/refractory (r/r) disease. The outcome is poor, with cure rates <30%, and there is no standard of care. Rituximab‐containing salvage regimens may provide a complete/partial response in 60–70% of cases. However, long‐term survival is <10% for non‐transplanted patients. Autologous or allogeneic haematopoietic stem cell transplant is, nowadays, the best option for responding patients, with survival rates around 50%. The benefit of autologous versus allogeneic HSCT is not clear. Numerous novel therapies for r/r B‐NHL are currently being tested in adults, including next‐generation monoclonal antibodies, novel cellular therapy strategies and therapies directed against new targets. Some are under investigation also in children/adolescents, with promising preliminary results.

中文翻译:

复发/难治性小儿侵袭性B细胞非霍奇金淋巴瘤的治疗。

侵略性B细胞非霍奇金淋巴瘤(B-NHL)占儿童/青少年NHL的约60%。在新近诊断的伯基特淋巴瘤和弥漫性大B细胞淋巴瘤中,短期强化多药化疗与五年无事件生存率约90%相关。极少有侵略性B-NHL的儿童/青少年表现出复发/难治性(r / r)疾病。结果差,治愈率<30%,没有标准的护理。含利妥昔单抗的挽救方案可能在60-70%的病例中提供完全/部分缓解。但是,非移植患者的长期生存率<10%。如今,自体或同种异体造血干细胞移植是应对患者的最佳选择,存活率约为50%。自体异基因HSCT尚不清楚。目前,许多针对R / r B-NHL的新疗法正在成人中进行测试,包括下一代单克隆抗体,新颖的细胞疗法和针对新靶标的疗法。一些儿童/青少年也正在接受调查初步结果令人鼓舞。
更新日期:2020-03-06
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