UPDATES ON HODGKIN DISEASE

Abstract

Hodgkin Lymphoma (HL) is a B-cell malignancy accounting for approximately 10% of all lymphoma cases and 5% of lymphoma-related mortalities. Incidence increases in younger adults and those above 55-years of age and has a bimodal distribution. Approximately 95% of all HL cases are diagnosed as classical Hodgkin Lymphoma (cHL) and 5% as Nodular Lymphocyte Predominant B-cell Lymphoma (NLPBL). Nodular sclerosis, mixed cellularity, lymphocyte depletion, and lymphocyte‐rich HL are subgroups of classical HL. Risk Stratification An accurate assessment of the stage of disease in patients with HL is critical for the selection of the appropriate therapy. Prognostic models that identify patients at low or high risk for recurrence, as well as the response to therapy as determined by Positron Emission Tomography (PET) scan, are used to optimize therapy. Risk‐Adapted Therapy Initial therapy for HL patients is based on the histology of the disease, the anatomical stage and the presence of poor prognostic features. Patients with early‐stage disease are typically treated with combined modality strategies utilizing abbreviated courses of combination chemotherapy followed by involved‐field radiation therapy, whereas those with advanced stage disease receive a longer course of chemotherapy often without radiation therapy. However, newer agents including brentuximab vedotin and anti‐PD‐1 antibodies are now standardly incorporated into frontline therapy. Management of Relapsed/Refractory Disease High‐Dose Chemotherapy (HDCT) followed by an Autologous Stem Cell Transplant (ASCT) is the standard of care for most patients who relapse following initial therapy. For patients who fail HDCT with ASCT, brentuximab vedotin, PD‐1 blockade, non‐myeloablative allogeneic transplant or participation in a clinical trial should be considered. The AETHERA study (NCT01100502) shows that Brentuximab Vedotin (BV) improves Progression-Free Survival (PFS) after ASCT in patients with Refractory or Relapsed HL (R/R HL). For patients who relapse after ASCT, BV, and anti-PD-1, monoclonal antibodies were considered incurable, and their outcome is rather dismal, with a median Overall Survival (OS) of 2-years. For Refractory or Relapsed cHL (R/R cHL) patients who have failed both ASCT and BV, Chimeric Antigen Receptor T-cell (CAR-T) therapy offers a new therapeutic option.