Revised Adult T-Cell Leukemia-Lymphoma International Consensus Meeting Report
Open accessAbstract
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
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AZT/IFN-α is not currently approved for ATL treatment in Japan, despite being the worldwide standard for acute, chronic, smouldering symptomatic and PCT-ATL subtypes.
“AZT/IFN-α is not currently approved for ATL treatment in Japan, despite being the worldwide standard for acute, chronic, smouldering symptomatic and PCT-ATL subtypes [194].”
From Current State of Therapeutics for HTLV-1 (2024) · cites this paper as [194]
The Introduction (page not provided) states that zidovudine and interferon-alpha are not approved in Japan but can be used elsewhere. The sections on aggressive ATL and the primary cutaneous variant (pages not provided) present AZT/IFN as a treatment option, not the worldwide standard for every listed subtype; the cutaneous-variant section also offers intensive chemotherapy. (LLM, conf=0.88)
Experimental — accuracy not yet measuredAggressive forms of ATL are treated with intensive chemotherapy, coupled with concurrent or low-dose azidothymidine and interferon-alpha (AZT/IFN-α) for maintenance.
“Aggressive forms of ATL are treated with intensive chemotherapy, coupled with concurrent or low-dose azidothymidine, an NRTI developed for HIV-AIDS, and interferon-alpha (AZT/IFN-α) for maintenance [194].”
From Current State of Therapeutics for HTLV-1 (2024) · cites this paper as [194]
The ‘ALLO-HSCT FOR AGGRESSIVE ATL’ section describes intensive chemotherapy and AZT/IFN as treatment options for aggressive ATL, but does not say they are routinely given together. The ‘ELDERLY AND NON–TRANSPLANTATION-SUITABLE PATIENTS WITH AGGRESSIVE ATL’ consensus statement allows AZT/IFN maintenance after less-intensive induction in selected patients; it does not establish concurrent AZT/IFN with intensive chemotherapy or specify low-dose AZT/IFN maintenance. (LLM, conf=0.87)
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Current State of Therapeutics for HTLV-1
Wang, T. T., Hirons, A., Doerflinger, M., Morris, K. V., Ledger, S., Purcell, D. F. J., Kelleher, A. D., & Ahlenstiel, C. L. (2024) · Viruses · doi:10.3390/v16101616 · Cited by 22 · see citing works
“Lesser-known medical burdens of HTLV-1 infection extend beyond this list, as patients report experiencing non-specific neuropathic/non-neuropathic pain alongside infection [262].”
BackgroundResultInfluential
- Authors
- Cook, L. B., Fuji, S., Hermine, O., Bazarbachi, A., Ramos, J. C., Ratner, L., Horwitz, S., Fields, P., Tanase, A., Bumbea, H., Cwynarski, K., Taylor, G., Waldmann, T. A., Bittencourt, A., Marcais, A., Suarez, F., Sibon, D., Phillips, A., Lunning, M., Farid, R., Imaizumi, Y., Choi, I., Ishida, T., Ishitsuka, K., Fukushima, T., Uchimaru, K., Takaori-Kondo, A., Tokura, Y., Utsunomiya, A., Matsuoka, M., Tsukasaki, K., & Watanabe, T.
- Affiliations
- National Health Service (GB)
- Osaka International Cancer Institute (JP)
- Hôpital Necker-Enfants Malades (FR)
- American University of Beirut (LB)
- University of Miami (US)
- Washington University in St. Louis (US)
- Memorial Sloan Kettering Cancer Center (US)
- St Thomas' Hospital (GB)
- Fundeni Clinical Institute, Bucharest, Romania
- Clinical Emergency Hospital Bucharest (RO)
- University College London (GB)
- Imperial College London (GB)
- National Institutes of Health (US)
- Universidade Federal da Bahia, Salvador, Brazil
- Necker University Hospital, Paris, France
- Weill Cornell Medicine (US)
- University of Nebraska at Omaha (US)
- Mashhad University of Medical Sciences (IR)
- Nagasaki University (JP)
- National Hospital Organization Kyushu Cancer Center (JP)
- Iwate Medical University (JP)
- Kagoshima University Hospital (JP)
- University of the Ryukyus (JP)
- The University of Tokyo (JP)
- Kyoto University, Kyoto, Japan
- Hamamatsu University (JP)
- Imamura Hospital (JP)
- Kumamoto University (JP)
- Saitama Medical University (JP)
- Corresponding author
- Tsukasaki, Kunihiro
- ORCID
- Cook, Lucy B.
- Fuji, Shigeo
- Hermine, Olivier
- Bazarbachi, Ali
- Ramos, Juan Carlos
- Ratner, Lee
- Fields, Paul
- Bumbea, Horia
- Cwynarski, Kate
- Taylor, Graham
- Waldmann, Thomas A.
- Sibon, David
- Phillips, Adrienne
- Lunning, Matthew
- Imaizumi, Yoshitaka
- Choi, Ilseung
- Ishida, Takashi
- Ishitsuka, Kenji
- Fukushima, Takuya
- Uchimaru, Kaoru
- Tokura, Yoshiki
- Utsunomiya, Atae
- Matsuoka, Masao
- Tsukasaki, Kunihiro
- Watanabe, Toshiki
- Venue
- Journal of Clinical Oncology
- Published
- 2019-03-10
- Volume
- 37
- Issue
- 8
- Pages
- 677-687
- ISSN
- 0732-183X (ISSN-L)
- 1527-7755
- PMC
- PMC6494249
- OpenAlex
- W2909144857
- Cited by
- 267
- References
- 2
- Citations by year
- 2026: 21 · 2025: 43 · 2024: 26 · 2023: 35 · 2022: 35 · 2021: 50 · 2020: 40 · 2019: 17
- FWCI
- 14.19
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- cc-by
- Locations
- Journal of Clinical Oncology (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- Spiral (Imperial College London) (submittedVersion, cc-by)
- Funders
- Japan Agency for Medical Research and Development (JP18ck0106338)
- National Institute for Health and Care Research
- National Institutes of Health (CA008748)
- Japan Society for the Promotion of Science (16H04713, 16H05248, 16H06277)
- National Cancer Institute (CA008748, R21CA234640, P30CA008748)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › T-cell and Retrovirus Studies
- Health Sciences › Medicine › Oncology › Viral-associated cancers and disorders
- Health Sciences › Medicine › Dermatology › Cutaneous lymphoproliferative disorders research
- Keywords
- adult T-cell leukemia/lymphoma, HTLV-1, cutaneous T-cell lymphoma, central nervous system lesions, elderly patients, allogeneic hematopoietic stem cell transplantation, novel agents, prognostic factors, treatment options, response criteria, molecular pathophysiology
- MeSH terms
- Antineoplastic Agents, Humans, Medical Oncology, Risk Factors, Skin Neoplasms, Transplantation, Homologous, Human T-lymphotropic virus 1, Leukemia-Lymphoma, Adult T-Cell, Central Nervous System Neoplasms, Treatment Outcome, Hematopoietic Stem Cell Transplantation, Consensus
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