From the Guidelines
Treatment for angioimmunoblastic T-cell lymphoma (AITL) should involve combination chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) administered every 21 days for 6 cycles, as it remains the most commonly used first-line regimen despite disappointing outcomes for patients with PTCL-NOS and AITL compared to DLBCL 1.
Key Considerations
- For younger, fit patients, consolidation with high-dose chemotherapy and autologous stem cell transplantation may improve long-term outcomes 1.
- Alternative regimens include CHOEP (CHOP plus etoposide) or dose-adjusted EPOCH (etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin), which have shown promise in certain subgroups of patients with T-cell lymphomas 1.
- The use of more intensive chemotherapy regimens has not resulted in favorable outcomes in patients with PTCL, with the exception of ALCL 1.
Relapsed or Refractory Disease
- Options for relapsed or refractory disease include gemcitabine-based regimens, bendamustine, or novel agents like the histone deacetylase inhibitor romidepsin (14 mg/m² on days 1,8, and 15 of a 28-day cycle) or the antifolate pralatrexate (30 mg/m² weekly for 6 weeks in 7-week cycles) 1.
- Targeted therapies showing promise include lenalidomide, brentuximab vedotin (for CD30+ cases), and IDH2 inhibitors like enasidenib for cases with IDH2 mutations.
- Allogeneic stem cell transplantation may be considered for eligible patients with chemosensitive relapsed disease.
Supportive Care
- AITL is challenging to treat due to its aggressive nature and immunodeficiency complications, with frequent infections requiring prophylactic antimicrobials and supportive care during treatment 1.
From the Research
Treatment Options for Angioimmunoblastic Lymphoma
- The treatment of angioimmunoblastic T-cell lymphoma (AITL) can be challenging due to its rarity and poor prognosis 2.
- Chemotherapy combined with radiotherapy has been shown to be effective in some cases, with a study reporting a patient who recovered well with no sign of tumor recurrence after undergoing eight cycles of CHOP (cyclophosphamide, hydroxydaunorubicin, oncovin, prednisone) followed by TOMO radiotherapy 2.
- The addition of rituximab to CHOP has been shown to improve overall and complete response rates, as well as event-free and overall survival in patients with aggressive non-Hodgkin's lymphoma, although its effectiveness in AITL specifically is not well established 3.
Salvage Therapies
- For patients with refractory or relapsed AITL, a combination of cyclosporine, prednisone, and high-dose intravenous immunoglobulin has been shown to be effective, with an overall response rate of 75% and a median progression-free survival of 25.5 months 4.
- Allogeneic stem cell transplantation may offer a chance for long-term remission in patients with relapsed or refractory AITL 5.
- Agents with preferential activity in relapsed or refractory AITL include epigenetic modifiers, such as histone deacetylase inhibitors and hypomethylating agents, as well as targeted agents like brentuximab vedotin and phosphoinositide-3-kinase inhibitors 5.
Emerging Therapies
- Cyclosporine has been identified as a potential therapeutic option for AITL, particularly in patients who are not candidates for hematopoietic stem cell transplantation, with an overall response rate of 86% in second or later lines 6.
- Further research is needed to establish the effectiveness of these emerging therapies and to develop more individualized treatment approaches for patients with AITL 5, 6.