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

Treatment of Symptomatic Smouldering Adult T-cell Leukemia-Lymphoma with Skin or Lung Lesions, Opportunistic Infections, or Chronic Disease

Adult T-cell leukemia-lymphoma (ATLL) encompasses several clinical subtypes that require distinct management strategies. Patients with symptomatic smouldering disease, primary cutaneous involvement with tumoural lesions, or chronic ATLL represent a specific group for whom a defined first-line treatment approach is recommended.

Clinical Scenario

This protocol covers: symptomatic smouldering ATLL with skin or lung lesions or opportunistic infections; primary cutaneous ATLL with tumoural skin lesions; and chronic ATLL.

Treatment Approach — Partial Overview

The recommended first-line strategy for these patients involves a combination of antiviral and immunomodulatory therapy. When skin lesions are present, skin-directed treatment is added to the systemic approach. Antimicrobial prophylaxis for opportunistic infections is incorporated for all patients in this group, with specific infectious considerations addressed as part of the complete protocol. The full regimen — including drug selection, eligibility criteria, prophylaxis specifications, and sequencing — is detailed in the complete protocol.

Instant Access to Structured Evidence-Based Regimens

References

  1. Zidovudine-interferon-a (IFN-a) can be recommended for patients with symptomatic smouldering (skin or lung lesions, opportunistic infections), primary cutaneous with tumoural lesions or chronic ATLL [III, B; not EMA or FDA approved], along with skin-directed therapy in case of skin lesions [III, B].
  2. Antimicrobial prophylaxis for opportunistic infections can be recommended for all patients [II, B].
  3. In case of positive Strongyloides stercoralis serology, treatment can be initiated even if the patient is asymptomatic [III, B].

DOI: 10.1016/j.annonc.2025.01.023

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