Treatment Options for Nodal Lymphomas of T Follicular Helper Cell Origin

Doxorubicin-based combination chemotherapy, such as the CHO(E)P regimen (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone), is commonly used for patients with AITL and other nodal lymphomas of TFH cell origin.[2,5] For CD30-positive cases, brentuximab vedotin combined with cyclophosphamide, doxorubicin, and prednisone is the proposed standard of care.[8][Level of evidence C3] Patients with AITL were included in a clinical trial involving mostly patients with anaplastic large cell lymphoma. A benefit for this smaller AITL subgroup cannot be established.[8,9][Level of evidence C3] For more information, see the Treatment of Anaplastic Large Cell Lymphoma section.

The International Peripheral T-Cell Lymphoma Project involving 22 international centers identified 243 patients with AITL. The 5-year overall survival rate was 33%, and the failure-free survival rate was 18%.[10] Myeloablative chemotherapy and radiation therapy with autologous or allogeneic peripheral stem cell support has been described in anecdotal reports.[11-16][Level of evidence C3] Anecdotal responses have been reported for patients who received cyclosporine,[17] pralatrexate,[18] bendamustine,[19] belinostat,[20] the histone deacetylase inhibitor romidepsin, hypomethylating agents (HMAs),[21,22] and brentuximab vedotin (even if there is little or no CD30 expression on the lymphoma).[23,24][Level of evidence C3] Occasional spontaneous remissions and protracted responses to steroids only have been reported. Given increased frequency of pathogenic variants in genes that regulate epigenetic modification—such as TET2, IDH2, and DNMT3A—there is increased interest in therapies targeting these aberrations with HMAs and histone deacetylase inhibitors with anecdotal reports of improved efficacy compared with other subsets of PTCL.[21,22,25][Level of evidence C3] Further studies are needed to better characterize the efficacy of this approach.[21,22,25]