Severe Cytokine Release Syndrome and Hemophagocytic Lymphohistiocytosis (HLH)-Like Syndrome Following Administration of Combined Brentuximab Vedotin and Nivolumab for Recurrent Classical Hodgkin Lymphoma: A Case Report

Brentuximab vedotin (BV) and nivolumab are increasingly utilized as a novel regimen in patients with relapsed/refractory classical Hodgkin lymphoma (cHL). A 26-year-old male presented to the hospital with refractory diabetic ketoacidosis and multiple electrolyte abnormalities, 9 days after the first dose of brentuximab vedotin and nivolumab for recurrent classical Hodgkin lymphoma. During his hospitalization, he developed multi-organ failure. His workup showed elevated cytokine levels concerning severe cytokine release syndrome (CRS) and hemophagocytic lymphohistiocytosis (HLH)-like syndrome. Despite treatment with CRS- and HLH-directed therapies, his clinical status deteriorated due to ongoing multifactorial shock and worsening multi-organ dysfunction, and comfort care measures were eventually pursued. To our knowledge, there have been no other cases reported of HLH-like syndrome after the combination of BV and nivolumab in patients with cHL. This case of a fatal adverse event following one dose of BV and nivolumab underscores the vital need for close monitoring of patients receiving this treatment regimen.