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

Treatment of Acute Adult T-cell Leukaemia-Lymphoma Without Bulky Lesions (Non-Lymphomatous Type)

This protocol addresses a well-defined subgroup: acute-type adult T-cell leukaemia-lymphoma (ATLL) that is non-lymphomatous in pattern and presents without bulky lesions — a combination of features that informs a specific treatment pathway, including first-line options suited to patients unsuitable for intensive approaches.

Clinical Scenario

Acute adult T-cell leukaemia-lymphoma; without bulky lesions; not lymphoma-type. This presentation is distinct from lymphoma-type and indolent ATLL subtypes and follows a dedicated management algorithm.

Treatment Approach (Partial Overview)

For relapsed or refractory disease in this setting, options can include a platinum-based second-line regimen or targeted monotherapy selected according to the individual tumour marker profile. In eligible patients who achieve a response, allogeneic haematopoietic stem-cell transplantation may also be considered.

Full selection criteria, sequencing logic, and eligibility requirements are available in the complete protocol below.

References

DOI: 10.1016/j.annonc.2025.01.023

High-dose zidovudine-IFN-a can be recommended as first-line therapy for patients with acute, non-bulky, non-lymphomatous ATLL whenever feasible, particularly for those who are unsuitable for intensive ChT or allo-HSCT [III, B; not EMA or FDA approved].

A second-line platinum-based regimen can be considered [IV, B].

Monotherapy with mogamulizumab (if CCR4-positive; not EMA or FDA approved), BV (if CD30-positive; not EMA or FDA approved), alemtuzumab (if CD52-positive; not EMA or FDA approved) or lenalidomide (not EMA or FDA approved) can be considered [III, B].

Allo-HSCT (if not used in first line) should be considered in HSCT-eligible responding patients [III, A].

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