From the Guidelines
The standard treatment for ALK-positive anaplastic large cell lymphoma (ALCL) is brentuximab vedotin 1.8 mg/kg IV every 3 weeks, in combination with CHP chemotherapy, as it has shown to be effective in improving overall survival and progression-free survival rates, as seen in the ECHELON-2 trial 1.
Treatment Options
- Brentuximab vedotin in combination with CHP chemotherapy is a preferred first-line therapy option for patients with ALCL, ALK-positive, due to its effectiveness in improving overall survival and progression-free survival rates 1.
- Multiagent chemotherapy regimens, such as CHOP or CHOEP, may also be considered, particularly for patients with stage I-II ALCL, ALK-positive, where 6 cycles of chemotherapy with or without involved-site radiation therapy (ISRT) is recommended 1.
- Autologous stem cell transplantation may be considered for eligible patients with high-risk or relapsed disease, as it has shown to improve overall survival rates in certain patient populations 1.
Disease Prognosis
- ALK-positive ALCL generally has a more favorable prognosis than ALK-negative disease, with 5-year survival rates of 70-90% 1.
- Young age and limited disease stage are additional positive prognostic factors, with patients under 60-65 years old showing improved overall survival rates with certain treatment regimens 1.
Treatment Monitoring
- Treatment response should be monitored with PET/CT scans, typically after 2-4 cycles and at the completion of therapy, to assess disease progression and response to treatment.
- Regular follow-up is essential after treatment completion, typically every 3-6 months for the first two years, then less frequently thereafter, to monitor for disease recurrence and manage any potential long-term side effects of treatment.
From the FDA Drug Label
1.5 Previously Untreated Systemic Anaplastic Large Cell Lymphoma (sALCL) or Other CD30-Expressing Peripheral T-cell Lymphomas (PTCL), in Combination with Chemotherapy ADCETRIS is indicated for the treatment of adult patients with previously untreated sALCL or other CD30-expressing PTCL, including angioimmunoblastic T-cell lymphoma and PTCL not otherwise specified (NOS), in combination with cyclophosphamide, doxorubicin, and prednisone. 1.6 Relapsed Systemic Anaplastic Large Cell Lymphoma (sALCL) ADCETRIS is indicated for the treatment of adult patients with sALCL after failure of at least one prior multi-agent chemotherapy regimen.
The treatment for Anaplastic Large Cell Lymphoma (ALCL), ALK positive is:
- Brentuximab vedotin (IV) in combination with chemotherapy for previously untreated patients
- Brentuximab vedotin (IV) for relapsed patients after failure of at least one prior multi-agent chemotherapy regimen 2 2 Key points:
- Brentuximab vedotin (IV) is indicated for the treatment of adult patients with previously untreated or relapsed Systemic Anaplastic Large Cell Lymphoma (sALCL)
- The recommended dosage of Brentuximab vedotin (IV) is 1.8 mg/kg up to a maximum of 180 mg, administered every 3 weeks 2
From the Research
Treatment Overview
- The standard first-line treatment for ALK-positive anaplastic large cell lymphoma (ALCL) consists of doxorubicin-containing polychemotherapy, which is associated with an overall response rate of ~90%, a 5-year relapse-free survival of ~60%, and a 5-year overall survival of 70% 3.
- Anthracycline-based chemotherapy regimens, including CHOP, CHOEP, or MACOP-B, have shown excellent results in treating ALK-positive ALCL 3, 4.
- Consolidative high-dose chemotherapy and autologous stem cell transplantation (HDC/ASCT) has been evaluated in patients in first remission with favorable results, but its superiority to standard chemotherapy is unproven and remains investigational 3.
Novel Therapies
- The development of novel therapies targeting CD30 and ALK appears promising, with agents such as brentuximab vedotin and crizotinib showing encouraging responses in relapsed ALCL 3, 4, 5, 6.
- Brentuximab vedotin, an anti-CD30 monoclonal antibody, has been associated with a high response rate and durable remissions in relapsed/refractory ALCL 6, 7.
- ALK inhibitors, such as crizotinib, alectinib, and ceritinib, are being used in clinical settings to treat ALK-positive ALCL 5.
Relapsed or Refractory Disease
- HDC/ASCT can effectively salvage a proportion of patients with relapsed or refractory ALK-positive ALCL 3, 4.
- Novel agents, such as brentuximab vedotin, are being investigated in the first-line setting and may improve overall responses and survival in both children and adults with ALK-positive ALCL 4, 6.