Key Points An accumulation of alterations in epigenetic modifiers and genes in the JAK/STAT pathway likely drives BI-ALCL oncogenesis. Whole exome sequencing of a large series of BI-ALCL demonstrates recurrent mutations in epigenetic regulators.

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Anti-programmed cell death protein 1 (anti-PD-1) antibodies demonstrated remarkable efficacy in patients with relapsed/ refractory (R/R) Hodgkin lymphoma (HL). In the 2 largest prospective studies that evaluated the anti-PD-1 antibodies nivolumab (CHECKMATE-205) 1 and pembrolizumab (KEYNOTE-087) 2 in R/R HL, the overall response rate and complete response (CR) rate were around 70% and 20%, respectively. These results led to the approval of nivolumab and pembrolizumab in R/R HL by the US Food and Drug Administration and the European Medicines Agency. Importantly, a significant proportion of HL patients in CR seem to experience durable remissions with anti-PD-1 treatment. 1 However, it is unclear whether these patients may be cured and how long they need to continue treatment with anti-PD-1 antibody. This is particularly important in HL patients because the remission rates are high, which raises questions about the need for continuing therapy. Most of these patients are young and may receive anti-PD-1 therapy indefinitely (or at least for a prolonged period), which could expose them to chronic or long-lasting toxicities and preclude procreation, and prolonged treatment has a high cost.5. Cheson BD, Horning SJ, Coiffier B, et al. Report of an international workshop to standardize response criteria for non-Hodgkin's lymphomas.