Hepatosplenic T cell lymphoma
ICD-10 C86.1 · ICD-11 2A90.8&XH8D49

Treatment of relapsed or refractory hepatosplenic T cell lymphoma

Clinical Scenario

Hepatosplenic T cell lymphoma (HSTCL) is an aggressive peripheral T cell malignancy. When disease fails to respond to or relapses after initial treatment, a structured salvage approach is required to guide subsequent management.

Treatment Approach — Partial Overview

Salvage strategies for relapsed or refractory HSTCL involve alternate induction regimens and, in selected cases, targeted immunotherapy guided by tumour marker expression. The complete regimen options, eligibility criteria, sequencing, and decision algorithm are set out in the full protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1182/bloodadvances.2025015857

Successful salvage in pediatric relapsed/refractory HSTCL is limited to case reports, using alternate induction regimens (eg, attempting ICE if CHOP was initially pursued), regimens common to mature B- and T-cell NHL (eg, dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin and hyper-cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with methotrexate and cytarabine), and consideration of targeted immunotherapy (eg, Bv for CD30+ cases and alemtuzumab for CD52+ cases).

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