Journal
CURRENT OPINION IN ONCOLOGY
Volume 33, Issue 5, Pages 406-411Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/CCO.0000000000000775
Keywords
allogeneic stem cell transplantation; hepatosplenic lymphoma; non-Hodgkin lymphoma; T-cell lymphoma; treatment
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Hepatosplenic lymphoma (HSTCL) is a rare T-cell malignancy with poor survival in young males, but recent studies suggest that allogeneic transplantation and specific chemotherapy may be effective treatment options. Recent molecular studies have identified potential targets for new therapeutic strategies.
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. Major progresses have been achieved in improving the outcome of HSTCL l patients using intensive chemotherapy and allogeneic transplantation.
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