Adult T-cell leukemia-lymphoma
ICD-10 C91.5 · ICD-11 2A90.5

Consolidation in Acute or Lymphoma-Type ATLL with Bulky Lesions After First-Line Chemotherapy Response

This protocol addresses patients with acute adult T-cell leukaemia-lymphoma or lymphoma-type adult T-cell leukaemia-lymphoma who present with bulky lesions and have achieved a chemosensitive response following first-line treatment. Consolidation of that response is the defining clinical objective.

First-line treatment in this setting consists of intensive combination chemotherapy — CHOP, CHOEP, or hyper-CVAD — with or without concurrent or sequential zidovudine plus interferon-α (when tolerated), alongside CNS and antimicrobial prophylaxis. The goal of that line is to achieve chemosensitive disease with an objective response. Patients who reach that milestone are candidates for consolidation under this protocol.

For chemosensitive patients, consolidation may involve allogeneic haematopoietic stem-cell transplantation, with specific measures incorporated into the conditioning regimen to address viral risk to donor cells. Patients ineligible for transplantation have distinct maintenance options. Full eligibility criteria, agent selection, and management of intolerance are detailed in the complete protocol.

Complete regimen, sequencing, and dosing details are available in the full structured protocol.

References

DOI: 10.1016/j.annonc.2025.01.023

Patients with acute or lymphoma-type ATLL with bulky lesions may receive intensive combination ChT [e.g. CHOP, CHOEP or hyperfractionated cyclophosphamide-vincristine-doxorubicin-dexamethasone-MTX-cytarabine (hyper-CVAD)] with or without concurrent or sequential zidovudine-IFN-α (if tolerated) [III, C; not EMA or FDA approved].

Chemosensitive patients can proceed to allo-HSCT [III, B].

An antiretroviral agent can be added to the conditioning regimen to prevent HTLV-1 neoinfection of donor cells [III, B].

Responding patients who are ineligible for allo-HSCT may receive maintenance therapy with zidovudine-IFN-α with or without arsenic trioxide [III, C; not EMA or FDA approved].

If zidovudine-IFN-α is poorly tolerated or there is no longer a response, oral low-dose etoposide may be considered as monotherapy or as part of a regimen [III, C].

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