With the advent of chimeric antigen receptors T-cell therapy, understanding their role in the development of hemophagocytic lymphohistiocytosis has become increasingly complex. We describe a case of a young patient with Epstein-Barr virus-positive diffuse large B-cell lymphoma, who was treated with axicabtagene ciloleucel. The patient developed progressive cytopenia and, on Day 73 post-infusion, met criteria for hemophagocytic lymphohistiocytosis. Bone marrow evaluation revealed hemophagocytosis without evidence of clonal B cells. The patient was treated with tocilizumab, dexamethasone, etoposide and anakinra, which eventually led to improvement. Unfortunately, the patient succumbed to an infection. Disease progression was confirmed posthumously.This case report explores the differential diagnosis of hyperinflammatory syndromes following chimeric antigen receptor T-cell therapy and highlights the reduced efficacy of this treatment in patients with a T-cell/histiocyte-rich background.
Keywords: CAR t-cell therapy; Epstein–Barr virus-positive large B-cell lymphoma; hemophagocytic lymphohistiocytosis; immune effector cell-associated hemophagocytic lymphohistiocytosis-like syndrome; post immune effector cells hyperinflammatory syndromes.
Lymphoma is an aggressive type of blood cancer that is typically treated with chemotherapy. However, the disease sometimes persists despite the initial treatment and requires new therapeutic options. A novel therapy that uses the patient’s own immune system, specifically a type of white blood cell called T lymphocytes, has recently been developed. These cells can be genetically modified to enhance recognition of cancer cells by expressing chimeric antigen receptors (CARs).CAR T-cell treatment is highly effective; however, it massively activates the patient’s immune system. This can lead to hyperinflammatory complications, including hemophagocytic lymphohistiocytosis, which is frequently fatal. This complication can arise from infections, cancer, genetic alterations or new immunotherapies.We present a case of hemophagocytic lymphohistiocytosis after CAR T-cell therapy, discuss the underlying biology, differential diagnosis and treatment options for this rare complication.