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首页> 外文期刊>Expert review of hematology >Relapsed/refractory primary mediastinal large B-cell lymphoma: a structured review of epidemiology, treatment guidelines and real-world treatment practices
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Relapsed/refractory primary mediastinal large B-cell lymphoma: a structured review of epidemiology, treatment guidelines and real-world treatment practices

机译：复发/难治原发性纵隔大型B细胞淋巴瘤：对流行病学的结构化审查，治疗指南和现实世界治疗措施

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Background: Primary mediastinal (thymic) large B-cell lymphoma (PMBCL) is an uncommon subtype of diffuse large B-cell lymphoma. Approximately 10-30% of patients experience refractory or relapsed PMBCL (rrPMBCL) after first-line therapy. Data and treatment guidelines for rrPMBCL are scarce, and management is based on clinical experience. Methods: Two structured literature reviews were undertaken to determine the incidence, prevalence, and mortality rates associated with rrPMBCL, and to identify clinical practice guidelines and real-world patterns of care. Results: Epidemiology studies included reported lymphomas (n = 1), non-Hodgkin lymphoma (n = 1), lymphoid neoplasm (n = 1), PMBCL (n = 6), and rrPMBCL (n = 1). Of 12 published treatment guidelines, only four provided recommendations for rrPMBCL. Sixteen studies provided data on real-world treatment patterns, but most were single-center studies with small patient numbers. Chemotherapy/immunochemotherapy, followed by high-dose treatment (HDT) and stem cell transplantation, was a mainstay of salvage therapy in most studies; real-world care generally followed treatment guidelines. Conclusions: Salvage chemotherapy (often with rituximab and radiotherapy), followed by HDT and stem cell transplantation, appears to be the standard real-world treatment for rrPMBCL. However, large prospective and retrospective studies are warranted to improve our knowledge of real-world treatment patterns.
机译：背景：原发性纵隔（胸腺）大B细胞淋巴瘤（PMBCL）是弥漫性大B细胞淋巴瘤的罕见亚型。大约10-30％的患者在一线疗法后经历难治性或复发的PMBCL（RRPMBCL）。 RRPMBCL的数据和治疗指南是稀缺的，管理层基于临床经验。方法：两项结构化文献综述，确定与RRPMBCL相关的发病率，患病率和死亡率，并确定临床实践指南和现实世界的护理模式。结果：流行病学研究包括报道淋巴瘤（n = 1），非霍奇金淋巴瘤（n = 1），淋巴肿瘤（n = 1），pMBCL（n = 6），和RRPMBCL（n = 1）。 12个公布的治疗准则，仅为RRPMBCL提供四项建议。十六项研究提供了关于现实世界治疗模式的数据，但大多数是单中心研究，具有小患者数量。化疗/免疫化学疗法，其次是高剂量治疗（HDT）和干细胞移植，是大多数研究中的救助治疗的主要效果;现实世界护理一般遵循治疗指南。结论：挽救化疗（通常用Rituximab和放射疗法），其次是HDT和干细胞移植，似乎是RRPMBCL的标准实际处理。然而，有必要提高大型前瞻性和回顾性研究，以提高我们对现实世界治疗模式的知识。

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