Adult T-cell leukemia-lymphoma: current treatment strategies and novel immunological approaches

Adult T-cell leukemia lymphoma (ATL) is a peripheral T-cell malignancy closely associated with human T-cell lymphotropic virus type I infection Clinically ATL is classified into four subtypes acute, lymphoma chronic and smoldering type Although the prognosis of chronic and smoldering-type ATL is relatively good that of patients with acute- or lymphoma-type ATL still remains extremely poor Zidovudine/IFN-alpha therapy seems to be promising although its efficacy has not yet been confirmed in well-designed prospective studies High-dose chemotherapy with the support of autologous transplantation does not improve outcome Allogeneic stem cell transplantation is promising and approximately 40% of aggressive ATL patients are expected to survive long-term although transplantation-related mortality is as high as 40-50% Stem cell transplantation using reduced-intensity conditioning is also effective and safer with graft-versus-ATL and graft-versus-human T-cell lymphotropic virus type I effects observed after transplantation Novel approaches including new agents such as purine nucleoside phosphorylase inhibitors and histone deacetylase inhibitors or targeted immunotherapy using antichemokine receptor-4 antibody or dendritic cell/peptide vaccine are also warranted