Anaplastic large cell lymphoma
ICD-10 C83.3 · ICD-11 2A90.A&XH9484, 2A90.B&XH9T74

First-Line Treatment of ALK-Positive Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma (ALCL) that is anaplastic lymphoma kinase (ALK)-positive is a biologically distinct subtype. Its first-line management is shaped by disease stage, bulk, and pre-therapeutic risk stratification.

Clinical Scenario

This protocol applies to patients with anaplastic large cell lymphoma confirmed to be ALK-positive, receiving first-line systemic therapy. Risk profile, stage, and disease bulk determine which treatment pathway is appropriate.

Treatment Approach

First-line management centres on chemotherapy — with regimens such as BV-CHP or CHOEP — and consolidation strategies whose scope and nature are guided by individual disease characteristics.

Full regimen selection, cycle details, consolidation criteria, and risk-adapted sequencing are in the complete protocol below.

Instant Access to Structured Evidence-Based Regimens

References

  1. First-line treatment of ALK-positive ALCL:
  2. In patients with limited-stage, non-bulky disease and a favourable pre-therapeutic risk profile, abbreviated ChT (e.g. three to four cycles of BV-CHP or CHOEP) [III, B] followed by consolidative ISRT (e.g. 30-40 Gy) in responding patients [IV, B] can be recommended.
  3. All other ChT-eligible patients should be offered six cycles of BV-CHP [I, A].
  4. Six cycles of CHO(E)P is an alternative for patients with stage I-II high-risk (bulky disease, IPI ≥2 or age >40 years) or advanced-stage disease [III, B].
  5. Consolidative ISRT can be recommended for patients with stage I-II, high-risk disease after six cycles of BV-CHP or CHO(E)P [IV, B].
  6. ASCT is generally not recommended for patients with stage III-IV low-risk disease [III, D].
  7. It can, however, be considered for patients with stage I-IV disease with high-risk features (e.g. IPI 3-4 and/or bulky extranodal disease and/or unconfirmed suspected residual disease activity) [II, B].

DOI: 10.1016/j.annonc.2025.07.014

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