Journal
JOURNAL OF CLINICAL ONCOLOGY
Volume 37, Issue 8, Pages 677-+Publisher
AMER SOC CLINICAL ONCOLOGY
DOI: 10.1200/JCO.18.00501
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Funding
- National Institute for Health Research Imperial Biomedical Research Centre
- National Institutes of Health, National Cancer Institute [P30-CA008748]
- Japan Agency for Medical Research and Development [JP18ak0101086h0001, JP18ck0106338]
- NATIONAL CANCER INSTITUTE [ZIASC004002] Funding Source: NIH RePORTER
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PurposeAdult T-cell leukemia-lymphoma (ATL) is a distinct mature T-cell malignancy caused by chronic infection with human T-lymphotropic virus type 1 with diverse clinical features and prognosis. ATL remains a challenging disease as a result of its diverse clinical features, multidrug resistance of malignant cells, frequent large tumor burden, hypercalcemia, and/or frequent opportunistic infection. In 2009, we published a consensus report to define prognostic factors, clinical subclassifications, treatment strategies, and response criteria. The 2009 consensus report has become the standard reference for clinical trials in ATL and a guide for clinical management. Since the last consensus there has been progress in the understanding of the molecular pathophysiology of ATL and risk-adapted treatment approaches.MethodsReflecting these advances, ATL researchers and clinicians joined together at the 18th International Conference on Human RetrovirologyHuman T-Lymphotropic Virus and Related Retrovirusesin Tokyo, Japan, March, 2017, to review evidence for current clinical practice and to update the consensus with a new focus on the subtype classification of cutaneous ATL, CNS lesions in aggressive ATL, management of elderly or transplantation-ineligible patients, and treatment strategies that incorporate up-front allogeneic hematopoietic stem-cell transplantation and novel agents.ResultsAs a result of lower-quality clinical evidence, a best practice approach was adopted and consensus statements agreed on by coauthors (> 90% agreement).ConclusionThis expert consensus highlights the need for additional clinical trials to develop novel standard therapies for the treatment of AT
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