Chemotherapy of Aggressive Adult T-cell Leukemia/Lymphoma (ATL)

Abstract

Treatment option for adult T-cell leukemia-lymphoma (ATL) is diverse, ranging from watchful waiting to intensive chemotherapy followed by allo-HSCT. A treatment strategy based on the clinical subtypes, prognostic factors, and response to initial therapy is suggested in an international consensus report (Tsukasaki et al. J Clin Oncol 27:453–459, 2009). Patients with acute, lymphoma, or unfavorable chronic subtypes (aggressive ATL) generally have a very poor prognosis due to multidrug resistance of ATL cells, a large tumor burden with multi-organ failure, hypercalcemia, and/or opportunistic infections. In the case of aggressive ATL, intensive chemotherapy such as VCAP-AMP-VECP (mLSG15) is usually recommended based on the results of a phase III trial (JCOG 9801) (Tsukasaki et al. J Clin Oncol 25:5458–5464, 2007). Other strategies include watchful waiting until disease progression for favorable chronic or smoldering ATL (indolent ATL), up-front allo-HSCT for relatively young patients with aggressive ATL, the combination of interferon alpha and zidovudine (IFN/AZT) for types with leukemic manifestation, and defucosylated humanized anti-CCR4 monoclonal antibody (mogamulizmab) for relapsed/refractory CCR4-positive aggressive ATL (Ishida et al. J Clin Oncol 30:837–842, 2012). In this chapter, chemotherapy of aggressive ATL will be reviewed with reference to consecutive trials by JCOG-LSG followed by addition of mogamulizmab in Japan (Tsukasaki et al. Jpn J Clin Oncol 42:85–95, 2012).