2019 Volume 8 Issue 1 Pages 9-15
Adult T-cell leukemia/lymphoma (ATLL) is an aggressive peripheral T-cell lymphoma with a dismal prognosis and is causally associated with human T-cell leukemia virus type 1. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the most effective treatment for patients with ATLL and can provide a chance of long-term remission through a graft-versus-ATLL effect. However, the incidence of relapse after allo-HSCT remains high at approximately 40%. Accumulating evidence shows that administering mogamulizumab and lenalidomide to patients with ATLL before or after allo-HSCT has drawbacks and advantages. Recent genomic and transcriptomic studies have shown that ATLL cells evade immune surveillance through mutated or hypermethylated genes encoding immune components and a novel regulatory mechanism of programmed death-ligand 1 (PD-L1) expression by PD-L1 3′-untranslated region disruption. Therefore, to overcome the poor prognosis of ATLL, further experimental and clinical research may lead to more effective and less toxic treatments before and after allo-HSCT. Research on immune-based therapies, such as vaccine and cell therapies against Tax or NY-ESO-1, is ongoing in Japan.
