Fertility preservation, imaging and radiotherapy guidelines, and best practices in relapse or salvage therapy for primary mediastinal B-cell lymphoma (PMBCL) are all highlighted in a new good practice paper from the British Society for Haematology.

Gene expression profiling data has shown PMBCL to be a separate clinicopathological entity with evidence of an overlap with classic Hodgkin lymphoma.

Biopsy, which should be reviewed by a hematopathologist, is required for a histological diagnosis of PMBCL. A multidisciplinary team should review the clinical presentation, pathology, and management plan.

Patients should receive positron emission tomography–computed tomography (PET/CT) at diagnosis, before steroids are administered, if possible, as standard of care. Results from the PET/CT should be reported in accordance with international guidelines.

Since patients with PMBCL are usually young adults at presentation, it’s important to consider fertility preservation in the face of chemotherapy.

The standard of care for treatment is six cycles of R-CHOP and involved site radiotherapy (ISRT). If the patient is being cared for at a site that can manage the complexities of dose adjustment and monitoring, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R) without ISRT is an alternative.