Hepatosplenic T-Cell Lymphomas
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.
KeywordsHepatosplenic T-cell lymphoma Immunosuppression Stem cell transplantation Genomic landscape
- 3.Vose J, Armitage J, Weisenburger D (2008) International TCLP. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol 26:4124–4130Google Scholar
- 13.Kotlyar DS, Osterman MT, Diamond RH et al (2011) A systematic review of factors that contribute to hepatosplenic T-cell lymphoma in patients with inflammatory bowel disease. Clin Gastroenterol Hepatol 9(36–41):e31Google Scholar
- 26.Wlodarska I, Martin-Garcia N, Achten R et al (2002) Fluorescence in situ hybridization study of chromosome 7 aberrations in hepatosplenic T-cell lymphoma: isochromosome 7q as a common abnormality accumulating in forms with features of cytologic progression. Genes Chromosom Cancer 33:243–251CrossRefGoogle Scholar
- 31.Voss MH, Lunning MA, Maragulia JC et al (2013) Intensive induction chemotherapy followed by early high-dose therapy and hematopoietic stem cell transplantation results in improved outcome for patients with hepatosplenic T-cell lymphoma: a single institution experience. Clin Lymphoma Myeloma Leuk 13:8–14CrossRefGoogle Scholar