Vol 55, No 3 (2024)
Review article
Published online: 2024-06-12

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Current knowledge and management of primary mediastinal large B-cell lymphoma

Sylwia Szydłowska1, Lidia Gil1, Grzegorz Dworacki2, Andrzej Balcerzak1
DOI: 10.5603/ahp.99320
Acta Haematol Pol 2024;55(3):137-144.

Abstract

Primary mediastinal large B-cell lymphoma (PMBCL) is a rare subtype of non-Hodgkin’s lymphoma that predominantly affects young females. Despite advances in the understanding of its biology, there is still no consensus on the optimal treatment strategy. First-line regimens such as R-CHOP and DA-EPOCH-R result in 2-year progression-free survival (PFS) rates of c.80% and overall survival (OS) rates of c.90%. However, the role of radiotherapy as a consolidation treatment remains unclear, with some studies suggesting limited benefits for patients with negative PET scans at the end of treatment. In cases of relapse or refractoriness, second-line therapies are comparable to those employed in the treatment of diffuse large B-cell lymphoma. Autologous stem cell transplantation remains a crucial salvage option, with 3-year OS and PFS rates of c.65% and 60%, respectively. New treatment approaches, including immune checkpoint inhibitors (e.g. pembrolizumab and nivolumab), chimeric antigen receptor-T (CAR-T) cell therapy, and bispecific antibodies, have demonstrated promising results. Further research into novel molecular targets and treatment combinations is necessary to improve clinical outcomes and minimize treatment-related toxicities in PMBCL.

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