From the Guidelines
Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive type of peripheral T-cell lymphoma that often expresses CD10, which serves as an important diagnostic marker to distinguish it from other T-cell lymphomas. According to the NCCN clinical practice guidelines in oncology, version 2.2022 1, AITL cells express T-cell–associated antigens and are usually CD4+, with expression of CXCL13 identified as a useful marker to help distinguish AITL from PTCL-NOS.
Key Characteristics of AITL
- AITL typically presents with generalized lymphadenopathy, hepatosplenomegaly, skin rash, and constitutional symptoms like fever and weight loss
- Patients often have immune dysregulation with hypergammaglobulinemia, autoimmune phenomena, and increased susceptibility to infections
- The initial paraffin panel for IHC studies may include pan–T-cell markers and can be expanded to include antibodies of T-cell lymphoma, if suspected, such as CD20, CD3, CD10, BCL6, Ki-67, CD5, CD30, CD2, CD4, CD8, CD7, CD56, CD21, CD23, TCRbeta, TCRdelta, PD1/CD279, ALK, and TP63 1
Treatment and Prognosis
- Treatment usually involves combination chemotherapy regimens such as CHOP (cyclophosphamide, doxorubicin, vincristine, prednisone) or more intensive approaches like CHOEP (CHOP plus etoposide)
- For eligible patients, consolidation with autologous stem cell transplantation may be considered in first remission due to the high relapse risk
- Newer targeted therapies including histone deacetylase inhibitors, phosphoinositide 3-kinase inhibitors, and immunotherapies are being investigated for refractory or relapsed disease
- The prognosis for AITL remains generally poor with 5-year survival rates of approximately 30-35%
From the Research
Angioimmunoblastic Lymphoma and CD10
- Angioimmunoblastic T-cell lymphoma (AITL) is a rare and complex lymphoproliferative disorder, characterized by widespread lymphadenopathy, extranodal disease, immune-mediated hemolysis, and polyclonal hypergammaglobulinemia 2.
- The diagnosis of AITL can be challenging due to the lack of specific clinical and histologic features, as well as the presence of morphologic mimickers.
- CD10 antibody has been reported as a potential diagnostic marker for AITL, but its utility is still being explored 3.
- A study of four cases of AITL found that CD10 antibody was negative in all cases, suggesting that its sensitivity as a diagnostic marker may be limited 3.
Treatment Approaches for AITL
- Conventional chemotherapy has not been effective in achieving durable responses in AITL, and the prognosis with current treatment approaches remains dismal 2.
- High-dose chemotherapy with autologous stem-cell support has been shown to be superior to standard chemotherapy regimens in adults with disseminated aggressive lymphoma 4.
- The role of CD10 in the diagnosis and treatment of AITL is still being investigated, and further studies are needed to determine its clinical significance 3.
- Other treatment approaches, such as rituximab-based regimens and allogeneic stem cell transplantation, may also be effective in treating AITL, but more research is needed to determine their efficacy 5, 6.