Relapsed/refractory primary mediastinal large B-cell lymphoma: a structured review of epidemiology, treatment guidelines and real-world treatment practices. Issue 3 (3rd March 2020)

• Record Type: Journal Article
• Authors: Takyar, Jitender; Raut, Monika; Borse, Rebekah; Balakumaran, Arun; Sehgal, Manu
• Is Part Of: Expert review of hematology. Volume 13:Issue 3(2020)
• Page Start: 275
• Page End: 287
• Publication Date: 2020-03-03
• Subjects: Epidemiology -- PMBCL -- practice guidelines -- primary mediastinal (thymic) large B-cell lymphoma -- treatment; Hematology -- Periodicals; 616.15005
• DOI: 10.1080/17474086.2020.1716725
• Languages: English
• ISSNs: 1747-4086

Abstract

ABSTRACT: 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.