Hepatosplenic T-cell lymphoma (HSTL) is a rare variant of extranodal peripheral T-cell lymphomas (PTCL), associated with aggressive disease course and a relentless track record for lethal outcomes. HSTL presents commonly in young men in their third or fourth decade. Of the known causes, immune dysregulation and immunosuppression are the key players in the pathogenesis of HSTL. Clinical manifestation includes hepatosplenomegaly, fevers, and weakness. Bone marrow involvement or organomegaly can cause cytopenias. Anthracycline-based regimens provide modest responses with most individuals dying within a year of diagnosis. Hematopoietic stem cell transplant (HSCT) can be offered to fit and eligible patients to prolong remissions. Disease relapse post chemotherapy has an aggressive phenotype, with limited salvage options available in the setting of declining performance status. Understanding the disease biology further to identify mechanistic-driven drug discovery could overcome the current limitations of existing therapeutic armamentarium.
Keywords: Genomic landscape; Hepatosplenic T-cell lymphoma; Immunosuppression; Stem cell transplantation.