A recent report demonstrates the promise of programmed death (PD-1) inhibition in improving outcomes for patients with a rare, difficult-to-treat type of lymphoma, published in The New England Journal of Medicine (July 6, 2017;377:89-91).
-----
Related Content
Lymphoma and Autologous Stem Cell Transplantation
FDA allows leukemia, lymphoma test marketing
-----
Gray zone lymphoma—a rare subtype that combines characteristics of Hodgkin lymphoma and primary mediastinal B-cell lymphoma—are often identified by frequent 9p24.1 copy-number alterations. Chemotherapy is only effective in approximately 50% of gray zone lymphoma cases, partly because of the aggressive nature of the disease and because of the difficulty of obtaining a correct diagnosis. Targeting the PD-1 pathway is a proposed method of treating gray zone lymphoma.
The published report describes three cases of patients receiving various PD-1 targeting drugs for their gray zone lymphoma. A young woman (age, 18 years) with such diagnosis demonstrated a partial response to dose-adjusted etoposide, doxorubicin, and cyclophosphamide with vincristine and prednisone plus rituximab (DA-EPOCH-R). Her disease progressed six weeks after salvage radiotherapy. Once treated with the immunotherapy agent pembrolizumab, she exhibited a complete metabolic response. She underwent allogeneic transplantation after 235 days of treatment and rearrangements of genes encoding the PD-1 and PD-2 ligands were detected by fluorescence in situ hybridization (FISH) analysis.
An older man (age, 76 years) with a gray zone lymphoma diagnosis also demonstrated a partial response to DA-EPOCH-R and subsequent disease progression. After treatment with pembrolizumab, the patient had a complete metabolic response and continues to be in remission on day 381 of treatment. FISH analysis detected amplification of PD-1 and PD-2 ligands, with 69% of cells having at least six copies per cell.
An older woman (age, 80 years) with a gray zone lymphoma diagnosis had a complete metabolic response to DA-EPOCH-R. However, she relapsed and showed disease progression after further treatment. Once treated with the immuno-agent nivolumab, she demonstrated a complete metabolic response and continues to be in remission on day 161 of treatment.
“These cases provide early evidence for using PD-1 inhibition in relapsed or refractory mediastinal gray zone lymphoma, which warrants further testing,” authors of the report wrote.—Zachary Bessette
