Primary cutaneous anaplastic large cell lymphoma
ICD-10 C86.6 · ICD-11 2B03.0&XH3400

Treatment of Primary Cutaneous ALCL Presenting with Multifocal Skin Lesions

Primary cutaneous anaplastic large cell lymphoma (PC-ALCL) presenting with multifocal skin lesions represents a distinct therapeutic scenario. The distribution and number of cutaneous lesions directly determine which evidence-based treatment strategy is most appropriate.

Clinical scenario: PC-ALCL with multifocal skin lesions — including patients with multiple papulonodular or relapsing cutaneous presentations. When lesions are multifocal, the treatment approach differs from that applied in single or very limited disease.

Treatment overview: For multifocal disease, the evidence base supports low-dose oral therapy as the primary approach. When only a few lesions are present, local radiotherapy is also an established option. Full regimen details — including specific agents, dosing, and sequencing — are available in the structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

  1. Patients presenting with multifocal skin lesions can best be treated with low-dose MTX, as in LyP [IV, A], or with RT [IV, A] in the case of only a few lesions.
  2. In the case of cosmetically disturbing lesions (e.g. scarring or many papulonodules), low-dose oral MTX (5–20 mg/week) and PUVA are the most effective therapies for reducing the number of skin lesions [IV, A].
  3. For patients with multifocal or relapsing skin lesions, a radiation dose of 8 Gy (2 × 4 Gy) was suggested.
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