Nasal type extranodal natural killer/T-cell lymphoma-associated hemophagocytic lymphohistiocytosis: a Case report and literature review

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome in which a large number of inflammatory cytokines, such as IL-6, TNF-α, and sCD25, are released into the blood circulation due to cytotoxic killing cells and dysfunction of natural killer (NK) cells. HLH can be induced by various infections, cancer, or hereditary disorders. HLH is usually fatal due to severe cytopenia and multiple organ failure. Nasal type extranodal natural killer/T-cell lymphoma (ENKTCL) is an aggressive non-Hodgkin’s lymphoma that is closely related to Epstein-Barr virus. ENKTCL can be complicated by HLH at initial diagnosis and recurrence. However, the optimal treatment strategies for ENKTCL-associated HLH are not well defined. Here, we report a case of ENKTCL-associated HLH that was successfully treated with liposomal doxorubicin, etoposide, methylprednisolone, and pegaspargase (DEPL regimen), followed by immunotherapy. This report aims to contribute to improved recognition, diagnosis, and treatment of lymphoma-associated HLH.

Keywords: Hemophagocytic lymphohistiocytosis, extranodal natural killer/T-cell lymphoma, liposomal doxorubicin, pegaspargase, lymphoma-associated HLH