Hepatosplenic T-cell lymphoma: treatment challenges

Curr Opin Oncol. 2021 Sep 1;33(5):406-411. doi: 10.1097/CCO.0000000000000775.


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.

Publication types

  • Review

MeSH terms

  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Liver Neoplasms* / therapy
  • Lymphoma, T-Cell* / drug therapy
  • Male
  • Splenic Neoplasms* / drug therapy
  • Transplantation, Homologous