Enteropathy associated T-cell lymphoma
ICD-10 C86.2 · ICD-11 2A90.7

What is the first-line treatment for Enteropathy-associated T-cell lymphoma?

Clinical context

This page covers the evidence-based first-line treatment approach for Enteropathy-associated T-cell lymphoma (EATL) at initial diagnosis. Multiple regimen strategies are considered, with patient fitness informing the choice of approach.

First-line approach — partial overview

Current evidence supports first-line combination chemotherapy, with consolidation using autologous stem cell transplantation considered in patients who are fit for intensive treatment. Several regimen pathways exist — the selection between them, their sequencing, and consolidation eligibility criteria are detailed in the full structured protocol.

Instant Access to Structured Evidence-Based Regimens

References

DOI: 10.1002/jha2.1049

Cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with or without etoposide consolidated by autologous stem cell transplantation (ASCT) are recommended for fit PTCL patients.

Patients are generally treated with cyclophosphamide, doxorubicin, vincristine, and prednisone—either with or without etoposide (CHO(E)P).

The strategy where one course of CHOP is followed by the IVE/MTX-regimen (ifosfamide/etoposide/epirubicin alternated with intermediate-dose MTX [3 g/m²]) followed by high-dose therapy – mostly carmustine/etoposide/cytarabine/melphalan (BEAM) – and ASCT has shown promising results in a case series of both 6 and 26 patients, where the 5-year progression-free survival (PFS) and OS were 52% and 60% respectively in the latter study.

A French study investigating CHP-BV followed by consolidation with ASCT as a frontline treatment for patients with EATL (n = 14) showed an overall response rate of 79% and those who responded all underwent ASCT.

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