Peripheral T-cell lymphoma, not otherwise specified

Peripheral T-cell lymphoma, not otherwise specified, is a broad category of biologically and clinically heterogeneous diseases, which can not be further classified into any other of the existing entities defined by the World Health Organization classification. Anthracycline-containing regimens, namely cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP), nowadays represent the standard first-line treatment; for patients who achieve a satisfactory response, a consolidation by means of autologous stem cell transplantation may offer a greater chance of long-term survival. Several patients, however, display treatment refractoriness or relapse soon after obtaining a response, and just a few of them are suitable transplant candidates. This is why several new agents, with innovative mechanisms of action, have been investigated in this context: pralatrexate, romidepsin, belinostat and brentuximab vedotin have been approved for relapsed and refractory peripheral T-cell lymphomas based on their activity, although they do not significantly impact on survival rates. The incorporation of such new drugs within a CHOP backbone is under investigation, in order to enhance response rates, allow a higher proportion of patients to be transplanted in remission and prolong survival. Blood First Edition Paper, prepublished online January 23, 2017; DOI 10.1182/blood-2016-08-692566 Copyright © 2017 American Society of Hematology For personal use only. on September 24, 2017. by guest www.bloodjournal.org From