What Is the First-Line Treatment for Hepatosplenic T Cell Lymphoma?
Hepatosplenic T cell lymphoma (HSTCL) is a rare, aggressive peripheral T cell malignancy. First-line management centres on prompt initiation of intensive induction therapy aimed at achieving deep remission before further consolidation.
Treatment Goal
The primary objective of induction is to achieve a complete remission (CR) or near-CR, which is the prerequisite for proceeding to consolidative treatment.
Treatment Approach — Partial Overview
The accepted approach is to initiate intensive, multiagent chemotherapy induction. Regimens incorporating platinum-based or cytarabine-containing combinations are among those used; antimetabolite-based therapy may also have a role, either alone or combined. Upfront splenectomy is not routinely recommended, though it may be considered in specific clinical circumstances.
Full regimen selection, sequencing criteria, and dosing are detailed in the complete structured protocol.
References
DOI: 10.1182/bloodadvances.2025015857
- The most accepted therapy paradigm is to initiate intensive, multiagent chemotherapy induction regimens.
- Commonly used induction regimens include those containing platinum agents (such as ICE in case 3) and cytarabine (such as IVAC [ifosfamide, etoposide, and cytarabine]), which may induce superior responses compared with anthracycline-containing regimens (CHOP [cyclophosphamide, doxorubicin, vincristine, and prednisone] or CHOP-like).
- Antimetabolite therapy (eg, pentostatin and pralatrexate) either as monotherapy or in combination with other agents may be effective.
- Of note, splenectomy is not necessary nor recommended upfront. It should be considered only in specific clinical scenarios or to support the start of treatment (eg, pain control, quality of life, or thrombocytopenia management).
- The goal of induction chemotherapy is to produce a CR or near-CR before proceeding with consolidative approaches, such as HSCT.
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