Hepatosplenic T cell lymphoma and hemophagocytic lymphohistiocytosis in an adult patient with Crohn’s disease on immunosuppressive therapy

Abstract

Hepatosplenic T cell lymphoma (HSTCL) is an exceedingly uncommon, aggressive peripheral T cell lymphoma comprising < 1% non-Hodgkin’s lymphomas (NHL). Despite treatments including allogeneic stem cell transplantation, median survival is < 2 years. In the majority of patients, the etiology of HSTCL is undetermined; although it has been associated with chronic immunosuppression which accounts for 20% of cases. HSTCL presents as a systemic illness, and sometimes in association with hemophagocytic lymphohistiocytosis syndrome (HLH). Our patient is a young male with a long-standing history of Crohn’s disease on immunosuppressive medications, who presented with progressive bicytopenia. He was diagnosed with HSTCL on a bone marrow biopsy and met clinical diagnostic criteria for HLH. He was started on chemotherapy and dexamethasone per HLH treatment protocol and underwent allogeneic hematopoietic stem cell transplantation (HSCT).