Treatment of Anaplastic Lymphoma
For anaplastic lymphoma, the treatment approach depends on the specific subtype, with brentuximab vedotin being the standard treatment for systemic anaplastic large cell lymphoma (sALCL) after failure of at least one prior multi-agent chemotherapy regimen, and for primary cutaneous anaplastic large cell lymphoma (pcALCL) after prior systemic therapy. 1
Primary Cutaneous CD30+ Anaplastic Large Cell Lymphoma (pcALCL)
Localized Disease (90% of cases)
- For solitary or localized tumors, radiotherapy or surgical excision is the first-line treatment 2
- Complete spontaneous remission may occur, requiring no further therapy 2
- Radiotherapy should be administered with electrons, with bolus, a 2 cm margin and a total dose of 20-30 Gy 2
- For patients with solitary or localized skin lesions, a total dose of 20 Gy in 8-10 fractions is effective and well-tolerated 2
Multifocal Disease (10% of cases)
- Low-dose methotrexate (5-20 mg/week) is recommended for patients with multifocal skin lesions 2
- Radiotherapy is appropriate when only a few lesions are present 2
- For multifocal or relapsing skin lesions, a radiation dose of 8 Gy (2 × 4 Gy) may be used 2
- Brentuximab vedotin should be considered in cases with multifocal skin lesions refractory to conventional therapies 2, 1
Advanced or Refractory Disease
- Brentuximab vedotin has shown high response rates in patients with primary cutaneous CD30+ lymphoproliferations 2, 1
- In a phase III trial, brentuximab vedotin showed an overall response rate of 75% and complete response rate of 31% in pcALCL patients 2
- Multi-agent chemotherapy is only indicated in patients presenting with or developing extracutaneous disease and in rare patients with rapidly progressive skin disease 2
Systemic Anaplastic Large Cell Lymphoma (sALCL)
First-line Treatment
- Multi-agent chemotherapy is the standard first-line treatment 3, 4
- CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) is the traditional first-line regimen 3
- For previously untreated sALCL, brentuximab vedotin in combination with cyclophosphamide, doxorubicin, and prednisone is FDA-approved 1
- For pediatric patients, intensive multi-agent regimens have shown good results with 65% overall survival at 5 years 5
Relapsed/Refractory Disease
- Brentuximab vedotin is FDA-approved for adult patients with sALCL after failure of at least one prior multi-agent chemotherapy regimen 1, 4
- High-dose chemotherapy followed by autologous stem cell transplantation (ASCT) may be considered for eligible patients with relapsed/refractory disease 6
- Sequential intensive treatment with chemotherapy +/- radiotherapy followed by ASCT has shown promising results in some studies 6
Special Considerations
Pediatric Anaplastic Lymphoma
- Multiagent chemotherapy cures approximately 60-75% of pediatric patients 7
- Relapse occurs in about 35% of cases 7
- For relapsed pediatric patients, options range from vinblastine monotherapy to hematopoietic stem cell transplantation 7
- New therapeutic approaches for refractory or high-risk forms include anti-CD30 monoclonal antibodies, ALK inhibitors, and CAR-T cells 7
Extranodal NK/T-cell Lymphoma, Nasal Type
- These lymphomas have an aggressive clinical course and should be treated with combined chemotherapy 2
- For patients presenting with a solitary skin lesion who are ineligible for systemic chemotherapy, radiotherapy should be considered 2
- In patients with stage I disease, radiotherapy is the first choice of treatment 2
Common Pitfalls and Caveats
- Misdiagnosis can lead to inappropriate treatment - ensure proper immunophenotypic and morphological diagnosis 2
- Comparison of treatment results across studies is challenging due to differences in diagnostic criteria 2
- Relapses in ALCL are frequent (up to 60%) and often occur early (mainly in first 24 months), requiring close follow-up 6
- Mediastinal and visceral involvement at presentation are predictive of increased risk of treatment failure in pediatric patients 5