LYMPHOMA: Edited by Dominique BronHepatosplenic T-cell lymphoma: treatment challengesBron, Dominiquea; De Leval, Laurenceb; Michiels, Sandraa; Wittnebel, Sebastiana Author Information aDepartment of Hematology, Institut Jules Bordet (ULB), Brussels, Belgium bInstitute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland Correspondence to Dominique Bron, Institut Jules Bordet (ULB), 1 Rue Héger Bordet, 1000 Brussels, Belgium. Tel: +32 2 541 32 32; e-mail: [email protected] Current Opinion in Oncology: September 2021 - Volume 33 - Issue 5 - p 406-411 doi: 10.1097/CCO.0000000000000775 Buy Metrics Abstract Purpose of review Hepatosplenic lymphoma (HSTCL) is a rare T-cell malignancy occurring in young males, associated with immune deficiency in 20% of the cases which, despite aggressive treatments, has a poor survival. Specific recommendations for first-line treatment remain debatable. Recent findings Published data covering case reports or series of HSTCL concur that allogeneic stem cell transplant should be proposed as a consolidation after response to chemotherapy in all patients eligible for transplant. In the light of two recent clinical examples, we also confirm that specific chemotherapy and a first-line consolidation with allogeneic transplantation when a donor is available to represent a treatment of choice these rare and distinctive lymphomas. Recent molecular studies are summarized in this review and suggest potential targets for new therapeutic strategies. Summary Major progresses have been achieved in improving the outcome of HSTCL l patients using intensive chemotherapy and allogeneic transplantation. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.