Revised Adult T-Cell Leukemia-Lymphoma International Consensus Meeting Report

Abstract

Purpose: Adult 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.

Methods: Reflecting these advances, ATL researchers and clinicians joined together at the 18th International Conference on Human Retrovirology-Human T-Lymphotropic Virus and Related Retroviruses-in 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.

Results: As a result of lower-quality clinical evidence, a best practice approach was adopted and consensus statements agreed on by coauthors (> 90% agreement).

Conclusion: This expert consensus highlights the need for additional clinical trials to develop novel standard therapies for the treatment of ATL.

FIG 1.

Primary cutaneous tumoral type (PCT) ATL. (A) Facial PCT with (B) histopathology that shows massive infiltration of pleomorphic lymphocytes in the dermis and subcutaneous tissue. (C) Nodulotumors of the chest with (D) histopathology that shows massive infiltration of atypical lymphocytes in the dermis and with epidermotropism.