Adult T-Cell Leukemia/Lymphoma

Abstract

Adult T-cell lymphoma/leukemia (ATL) is a rare T-cell lymphoproliferative neoplasm caused by human T-lymphotrophic virus 1. In its more common, aggressive forms, ATL carries one of the poorest prognoses of the non-Hodgkin lymphomas. The disease has clinical subtypes (ie, acute, lymphoma, chronic, and smoldering forms) defined by the presenting features, and therefore, the clinical course can vary. For the smoldering and lower-risk chronic forms, combinations involving antiviral therapies have shown some success. However, in many patients, the more indolent forms will evolve into the more aggressive subtypes. In the more aggressive acute, lymphoma, and higher-risk chronic forms, the literature supports initial treatment with combination chemotherapy followed by allogeneic transplantation as a potentially curative approach. Recently, mogamulizumab and lenalidomide have shown promise in the treatment of ATL. With better understanding of the molecular drivers of this disease, we hope that the therapeutic landscape will continue to expand.

Fig 1.

(A) Lymph node biopsy and (B) peripheral smear in adult T-cell lymphoma/leukemia (ATL). The neoplastic cells in ATL characteristically have a flower-like or clover-shaped appearance. By flow cytometry, they express CD2, CD4, CD5, CD45RO, CD29, and T-cell receptor αβ and do not express CD7, CD8, or CD26. CD3 expression is often reduced. Photographs courtesy of Ahmet Dogan.