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Acute Adult T-Cell Leukemia/Lymphoma

MedGen UID:
231024
•Concept ID:
C1332146
•
Neoplastic Process
Synonym: Acute Adult T-Cell Lymphoma/Leukemia

Definition

The most common variant of adult T-cell leukemia/lymphoma. It is characterized by systemic disease with a leukemic phase, generalized lymphadenopathy, and skin lesions. Hypercalcemia is commonly seen. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Nelarabine: when and how to use in the treatment of T-cell acute lymphoblastic leukemia.
Shimony S, DeAngelo DJ, Luskin MR
Blood Adv 2024 Jan 9;8(1):23-36. doi: 10.1182/bloodadvances.2023010303. PMID: 37389830Free PMC Article
Evolving therapy of adult acute lymphoblastic leukemia: state-of-the-art treatment and future directions.
Samra B, Jabbour E, Ravandi F, Kantarjian H, Short NJ
J Hematol Oncol 2020 Jun 5;13(1):70. doi: 10.1186/s13045-020-00905-2. PMID: 32503572Free PMC Article
Adult Acute Lymphoblastic Leukemia: Treatment and Management Updates.
Gregory S
Semin Oncol Nurs 2019 Dec;35(6):150951. Epub 2019 Nov 20 doi: 10.1016/j.soncn.2019.150951. PMID: 31759820

Recent clinical studies

Etiology

Human T-cell lymphotropic virus type 1 (HTLV-1) and lymphoid malignancies in Dominica: a seroprevalence study.
Adedayo OA, Shehu SM
Am J Hematol 2004 Dec;77(4):336-9. doi: 10.1002/ajh.20208. PMID: 15551358
HTLV-I positive adult T-cell leukaemia-lymphoma: report of a typical case from Italy.
Fanin R, Minutillo S, D'Agaro P, Raspadori D, Tassinari A, Tazzari P, Testoni N, Damiani D, Gallizia C, Michieli M
Haematologica 1990 Jul-Aug;75(4):375-80. PMID: 1980480

Diagnosis

Small RNA Profiling in an HTLV-1-Infected Patient with Acute Adult T-Cell Leukemia-Lymphoma at Diagnosis and after Maintenance Therapy: A Case Study.
Pessôa R, de Souza DRV, Nukui Y, Pereira J, Fernandes LA, Marcusso RN, de Oliveira ACP, Casseb J, da Silva Duarte AJ, Sanabani SS
Int J Mol Sci 2023 Jun 26;24(13) doi: 10.3390/ijms241310643. PMID: 37445821Free PMC Article
Welder's pulmonary hemosiderosis associated with systemic iron overload following exacerbation of acute adult T-cell leukemia/lymphoma.
Imoto N, Shiraki A, Furukawa K, Tange N, Murase A, Hayakawa M, Iwata Y, Kosugi H
J Clin Exp Hematop 2017 Oct 12;57(2):74-78. Epub 2017 Sep 6 doi: 10.3960/jslrt.17024. PMID: 28883221Free PMC Article
Adult T-cell leukemia/lymphoma in an adolescent presenting with skin lesions.
Lucas CT, Gillis KJ, Ness JM, Hammers YA, Crawford DF, Kelly DR, Theos A
Pediatr Dermatol 2008 May-Jun;25(3):373-7. doi: 10.1111/j.1525-1470.2008.00686.x. PMID: 18577047
Unusual relapse of adult T-cell leukemia/lymphoma after spontaneous remission.
Kawada H, Fukuda R, Suzuki M, Yamashita K, Matsuoka H, Hotta T
Leuk Res 1998 Feb;22(2):197-9. doi: 10.1016/s0145-2126(97)00150-1. PMID: 9593477
Acute adult T-cell leukemia/lymphoma presenting with florid cutaneous disease.
Wright SA, Rothe MJ, Sporn J, Van Voorhees AS, Grant-Kels JM
Int J Dermatol 1992 Aug;31(8):582-7. doi: 10.1111/j.1365-4362.1992.tb02725.x. PMID: 1428453

Therapy

Rapidly fatal HTLV-1-associated T-cell leukemia/lymphoma in a patient with SLE.
Fritsch-Stork RD, Leguit RJ, Derksen RH
Nat Rev Rheumatol 2009 May;5(5):283-7. doi: 10.1038/nrrheum.2009.49. PMID: 19412195
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
HTLV-I positive adult T-cell leukaemia-lymphoma: report of a typical case from Italy.
Fanin R, Minutillo S, D'Agaro P, Raspadori D, Tassinari A, Tazzari P, Testoni N, Damiani D, Gallizia C, Michieli M
Haematologica 1990 Jul-Aug;75(4):375-80. PMID: 1980480

Prognosis

Welder's pulmonary hemosiderosis associated with systemic iron overload following exacerbation of acute adult T-cell leukemia/lymphoma.
Imoto N, Shiraki A, Furukawa K, Tange N, Murase A, Hayakawa M, Iwata Y, Kosugi H
J Clin Exp Hematop 2017 Oct 12;57(2):74-78. Epub 2017 Sep 6 doi: 10.3960/jslrt.17024. PMID: 28883221Free PMC Article
Rapidly fatal HTLV-1-associated T-cell leukemia/lymphoma in a patient with SLE.
Fritsch-Stork RD, Leguit RJ, Derksen RH
Nat Rev Rheumatol 2009 May;5(5):283-7. doi: 10.1038/nrrheum.2009.49. PMID: 19412195
Adult T-cell leukemia/lymphoma in an adolescent presenting with skin lesions.
Lucas CT, Gillis KJ, Ness JM, Hammers YA, Crawford DF, Kelly DR, Theos A
Pediatr Dermatol 2008 May-Jun;25(3):373-7. doi: 10.1111/j.1525-1470.2008.00686.x. PMID: 18577047
Experimental acute adult T cell leukemia-lymphoma is associated with thymic atrophy in human T cell leukemia virus type I infection.
Simpson RM, Zhao TM, Hubbard BS, Sawasdikosol S, Kindt TJ
Lab Invest 1996 Mar;74(3):696-710. PMID: 8600320
Acute adult T-cell leukemia/lymphoma presenting with florid cutaneous disease.
Wright SA, Rothe MJ, Sporn J, Van Voorhees AS, Grant-Kels JM
Int J Dermatol 1992 Aug;31(8):582-7. doi: 10.1111/j.1365-4362.1992.tb02725.x. PMID: 1428453

Clinical prediction guides

Unusual relapse of adult T-cell leukemia/lymphoma after spontaneous remission.
Kawada H, Fukuda R, Suzuki M, Yamashita K, Matsuoka H, Hotta T
Leuk Res 1998 Feb;22(2):197-9. doi: 10.1016/s0145-2126(97)00150-1. PMID: 9593477
Experimental acute adult T cell leukemia-lymphoma is associated with thymic atrophy in human T cell leukemia virus type I infection.
Simpson RM, Zhao TM, Hubbard BS, Sawasdikosol S, Kindt TJ
Lab Invest 1996 Mar;74(3):696-710. PMID: 8600320
HTLV-I positive adult T-cell leukaemia-lymphoma: report of a typical case from Italy.
Fanin R, Minutillo S, D'Agaro P, Raspadori D, Tassinari A, Tazzari P, Testoni N, Damiani D, Gallizia C, Michieli M
Haematologica 1990 Jul-Aug;75(4):375-80. PMID: 1980480

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