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

Hepatosplenic T-cell lymphoma: treatment challenges

Dominique Bron  1 , Laurence De Leval  2 , Sandra Michiels  1 , Sebastian Wittnebel  1 ; EuroBloodNet for rare diseases
Affiliations
Review

Hepatosplenic T-cell lymphoma: treatment challenges

Dominique Bron et al. Curr Opin Oncol. .

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.

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