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Smoldering Adult T-Cell Leukemia/Lymphoma

MedGen UID:
234795
•Concept ID:
C1336015
•
Neoplastic Process
Synonym: Smoldering Adult T-Cell Lymphoma/Leukemia

Definition

An adult T-cell leukemia/lymphoma characterized by less than 5% neoplastic lymphocytes in the peripheral blood, absence of lymphadenopathy and hypercalcemia, and occasional skin or pulmonary lesions. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmoldering Adult T-Cell Leukemia/Lymphoma

Professional guidelines

PubMed

Diagnosis and management of adult T-cell leukemia/lymphoma.
Ishitsuka K
Semin Hematol 2021 Apr;58(2):114-122. Epub 2021 Mar 3 doi: 10.1053/j.seminhematol.2021.02.005. PMID: 33906721
Treatment advances and prognosis for patients with adult T-cell leukemia-lymphoma.
Katsuya H, Ishitsuka K
J Clin Exp Hematop 2017 Dec 27;57(3):87-97. Epub 2017 Jun 8 doi: 10.3960/jslrt.17008. PMID: 28592744Free PMC Article
Recent advances in the treatment of adult T-cell leukemia-lymphomas.
Utsunomiya A, Choi I, Chihara D, Seto M
Cancer Sci 2015 Apr;106(4):344-51. Epub 2015 Mar 6 doi: 10.1111/cas.12617. PMID: 25613789Free PMC Article

Recent clinical studies

Etiology

Pre-morbid human T-lymphotropic virus type I proviral load, rather than percentage of abnormal lymphocytes, is associated with an increased risk of aggressive adult T-cell leukemia/lymphoma.
Hodson A, Laydon DJ, Bain BJ, Fields PA, Taylor GP
Haematologica 2013 Mar;98(3):385-8. Epub 2012 Aug 8 doi: 10.3324/haematol.2012.069476. PMID: 22875625Free PMC Article
Combination of skin-directed therapy and oral etoposide for smoldering adult T-cell leukemia/lymphoma with skin involvement.
Sawada Y, Shimauchi T, Yamaguchi T, Okura R, Hama-Yamamoto K, Fueki-Yoshioka H, Ohmori S, Yamada S, Yoshizawa M, Hiromasa K, Tajiri M, Kabashima-Kubo R, Yoshioka M, Sugita K, Yoshiki R, Hino R, Kobayashi M, Izu K, Nakamura M, Tokura Y
Leuk Lymphoma 2013 Mar;54(3):520-7. Epub 2012 Aug 21 doi: 10.3109/10428194.2012.715351. PMID: 22830614

Diagnosis

Isolated cardiac valve involvement in smoldering adult T-cell leukemia/lymphoma.
Aguilar C, Beltran BE, Morales D, Gutiérrez-Garibay M, Villela L, Marques-Piubelli ML, Vega F, Miranda RN, Malpica L
Cardiovasc Pathol 2023 May-Jun;64:107513. Epub 2022 Dec 23 doi: 10.1016/j.carpath.2022.107513. PMID: 36567046
Assessing smoldering adult T cell leukemia/lymphoma: a case report.
Olayinka JT, Heilman E, Vatanchi M
Dermatol Online J 2021 Sep 15;27(9) doi: 10.5070/D327955137. PMID: 34755979
Primary cutaneous smoldering adult T-cell leukemia/ lymphoma.
Gittler J, Martires K, Terushkin V, Brinster N, Ramsay D
Dermatol Online J 2016 Dec 15;22(12) PMID: 28329538
Meeting report on the possible proposal of an extranodal primary cutaneous variant in the lymphoma type of adult T-cell leukemia-lymphoma.
Tsukasaki K, Imaizumi Y, Tokura Y, Ohshima K, Kawai K, Utsunomiya A, Amano M, Watanabe T, Nakamura S, Iwatsuki K, Kamihira S, Yamaguchi K, Shimoyama M
J Dermatol 2014 Jan;41(1):26-8. doi: 10.1111/1346-8138.12374. PMID: 24438141
Pre-morbid human T-lymphotropic virus type I proviral load, rather than percentage of abnormal lymphocytes, is associated with an increased risk of aggressive adult T-cell leukemia/lymphoma.
Hodson A, Laydon DJ, Bain BJ, Fields PA, Taylor GP
Haematologica 2013 Mar;98(3):385-8. Epub 2012 Aug 8 doi: 10.3324/haematol.2012.069476. PMID: 22875625Free PMC Article

Therapy

Assessing smoldering adult T cell leukemia/lymphoma: a case report.
Olayinka JT, Heilman E, Vatanchi M
Dermatol Online J 2021 Sep 15;27(9) doi: 10.5070/D327955137. PMID: 34755979
Combination of skin-directed therapy and oral etoposide for smoldering adult T-cell leukemia/lymphoma with skin involvement.
Sawada Y, Shimauchi T, Yamaguchi T, Okura R, Hama-Yamamoto K, Fueki-Yoshioka H, Ohmori S, Yamada S, Yoshizawa M, Hiromasa K, Tajiri M, Kabashima-Kubo R, Yoshioka M, Sugita K, Yoshiki R, Hino R, Kobayashi M, Izu K, Nakamura M, Tokura Y
Leuk Lymphoma 2013 Mar;54(3):520-7. Epub 2012 Aug 21 doi: 10.3109/10428194.2012.715351. PMID: 22830614
Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy.
Aouba A, Lambotte O, Vasiliu V, Divine M, Valensi F, Varet B, Bazarbachi A, Hermine O
Am J Hematol 2004 Jun;76(2):187-9. doi: 10.1002/ajh.20065. PMID: 15164389

Prognosis

Primary cutaneous smoldering adult T-cell leukemia/ lymphoma.
Gittler J, Martires K, Terushkin V, Brinster N, Ramsay D
Dermatol Online J 2016 Dec 15;22(12) PMID: 28329538
Meeting report on the possible proposal of an extranodal primary cutaneous variant in the lymphoma type of adult T-cell leukemia-lymphoma.
Tsukasaki K, Imaizumi Y, Tokura Y, Ohshima K, Kawai K, Utsunomiya A, Amano M, Watanabe T, Nakamura S, Iwatsuki K, Kamihira S, Yamaguchi K, Shimoyama M
J Dermatol 2014 Jan;41(1):26-8. doi: 10.1111/1346-8138.12374. PMID: 24438141
Pre-morbid human T-lymphotropic virus type I proviral load, rather than percentage of abnormal lymphocytes, is associated with an increased risk of aggressive adult T-cell leukemia/lymphoma.
Hodson A, Laydon DJ, Bain BJ, Fields PA, Taylor GP
Haematologica 2013 Mar;98(3):385-8. Epub 2012 Aug 8 doi: 10.3324/haematol.2012.069476. PMID: 22875625Free PMC Article
Combination of skin-directed therapy and oral etoposide for smoldering adult T-cell leukemia/lymphoma with skin involvement.
Sawada Y, Shimauchi T, Yamaguchi T, Okura R, Hama-Yamamoto K, Fueki-Yoshioka H, Ohmori S, Yamada S, Yoshizawa M, Hiromasa K, Tajiri M, Kabashima-Kubo R, Yoshioka M, Sugita K, Yoshiki R, Hino R, Kobayashi M, Izu K, Nakamura M, Tokura Y
Leuk Lymphoma 2013 Mar;54(3):520-7. Epub 2012 Aug 21 doi: 10.3109/10428194.2012.715351. PMID: 22830614

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