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).

Keywords

Hemophagocytic lymphohistiocytosis / Hepatosplenic T cell lymphoma / Crohn’s disease / Ferritin

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