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Leukemia-Lymphoma, Adult T-Cell

MedGen UID:
44128
Concept ID:
C0023493
Neoplastic Process
Synonyms: Adult T-Cell Leukemia; Adult T-Cell Leukemia-Lymphoma; Adult T-Cell Leukemia-Lymphomas; Adult T-Cell Leukemias; ATLL; HTLV Associated Leukemia Lymphoma; HTLV I Associated T Cell Leukemia Lymphoma; HTLV-Associated Leukemia-Lymphoma; HTLV-Associated Leukemia-Lymphomas; HTLV-I-Associated T-Cell Leukemia-Lymphoma; HTLV-I-Associated T-Cell Leukemia-Lymphomas; Human T Cell Leukemia Lymphoma; Human T Lymphotropic Virus Associated Leukemia Lymphoma; Human T Lymphotropic Virus-Associated Leukemia-Lymphoma; Human T-Cell Leukemia-Lymphoma; Human T-Cell Leukemia-Lymphomas; Leukemia Lymphoma, Adult T Cell; Leukemia Lymphoma, T Cell, Acute, HTLV I Associated; Leukemia, Adult T Cell; Leukemia, Adult T-Cell; Leukemia-Lymphoma, HTLV-Associated; Leukemia-Lymphoma, HTLV-I-Associated T-Cell; Leukemia-Lymphoma, Human T-Cell; Leukemia-Lymphoma, T-Cell, Acute, HTLV-I-Associated; Leukemia-Lymphomas, Adult T-Cell; Leukemia-Lymphomas, HTLV-Associated; Leukemia-Lymphomas, HTLV-I-Associated T-Cell; Leukemia-Lymphomas, Human T-Cell; Leukemias, Adult T-Cell; T Cell Leukemia Lymphoma, Adult; T Cell Leukemia Lymphoma, HTLV I Associated; T Cell Leukemia, Adult; T-Cell Leukemia, Adult; T-Cell Leukemia-Lymphoma, Adult; T-Cell Leukemia-Lymphoma, HTLV-I-Associated; T-Cell Leukemia-Lymphoma, Human; T-Cell Leukemia-Lymphomas, Adult; T-Cell Leukemia-Lymphomas, HTLV-I-Associated; T-Cell Leukemia-Lymphomas, Human; T-Cell Leukemias, Adult
SNOMED CT: Adult T-cell leukemia/lymphoma (77430005); Adult T-cell lymphoma/leukemia (77430005); Adult T-cell lymphoma (77430005); Adult T-cell leukemia (77430005); Adult T-cell leukemia/lymphoma (HTLV-1 positive) (77430005); Adult T-cell leukemia/lymphoma (110007008); Adult T-cell leukemia/lymphoma (clinical) (110007008); Adult T-cell lymphoma/leukemia (HTLV-1 +) (77430005); Adult T-cell leukemia (110007008)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019471
Orphanet: ORPHA86875

Definition

A rare, virus associated tumor due to human T-cell leukemia virus type 1 or human T-cell lymphotropic virus type 1 (HTLV-1) and is characterized by the presence of anti-HTLV-1 antibodies, and malignant, mature, medium-sized T cells with condensed chromatin and polylobated nuclei. The malignant cells exhibit a mature CD4+ T cells phenotype and express CD2, CD5, CD25, CD45RO, HLA-DR, and T-cell receptor αβ. Presentation is heterogeneous and is typically of aggressive leukemia or lymphoma, variable skin eruptions, and visceral organ involvement. [from ORDO]

Professional guidelines

PubMed

Watanabe K, Gomez AM, Kuramitsu S, Siurala M, Da T, Agarwal S, Song D, Scholler J, Rotolo A, Posey AD, Rook AH, Haun PL, Ruella M, Young RM, June CH
Blood Adv 2023 Jul 25;7(14):3416-3430. doi: 10.1182/bloodadvances.2022008327. PMID: 37058474Free PMC Article
Ishitsuka K
Int J Hematol 2023 Apr;117(4):473-474. Epub 2023 Mar 14 doi: 10.1007/s12185-023-03573-3. PMID: 36918503
Cook LB, Fuji S, Hermine O, Bazarbachi A, Ramos JC, Ratner L, Horwitz S, Fields P, Tanase A, Bumbea H, Cwynarski K, Taylor G, Waldmann TA, 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
J Clin Oncol 2019 Mar 10;37(8):677-687. Epub 2019 Jan 18 doi: 10.1200/JCO.18.00501. PMID: 30657736Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

Recent clinical studies

Etiology

Stuver R, Horwitz SM, Epstein-Peterson ZD
Curr Treat Options Oncol 2023 Aug;24(8):948-964. Epub 2023 Jun 10 doi: 10.1007/s11864-023-01111-1. PMID: 37300656Free PMC Article
Kameda T, Shide K, Tahira Y, Sekine M, Sato S, Ishizaki J, Takeuchi M, Akizuki K, Kamiunten A, Shimoda H, Toyama T, Maeda K, Yamashita K, Kawano N, Kawano H, Hidaka T, Yamaguchi H, Kubuki Y, Kitanaka A, Matsuoka H, Shimoda K
Viruses 2022 Mar 29;14(4) doi: 10.3390/v14040710. PMID: 35458440Free PMC Article
Miyashiro D, Sanches JA
Semin Diagn Pathol 2020 Mar;37(2):81-91. Epub 2019 Jul 30 doi: 10.1053/j.semdp.2019.07.010. PMID: 31387753
Cook LB, Fuji S, Hermine O, Bazarbachi A, Ramos JC, Ratner L, Horwitz S, Fields P, Tanase A, Bumbea H, Cwynarski K, Taylor G, Waldmann TA, 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
J Clin Oncol 2019 Mar 10;37(8):677-687. Epub 2019 Jan 18 doi: 10.1200/JCO.18.00501. PMID: 30657736Free PMC Article
Mehta-Shah N, Ratner L, Horwitz SM
J Oncol Pract 2017 Aug;13(8):487-492. doi: 10.1200/JOP.2017.021907. PMID: 28796966Free PMC Article

Diagnosis

Stuver R, Horwitz SM, Epstein-Peterson ZD
Curr Treat Options Oncol 2023 Aug;24(8):948-964. Epub 2023 Jun 10 doi: 10.1007/s11864-023-01111-1. PMID: 37300656Free PMC Article
Ishitsuka K
Semin Hematol 2021 Apr;58(2):114-122. Epub 2021 Mar 3 doi: 10.1053/j.seminhematol.2021.02.005. PMID: 33906721
Munakata W, Tobinai K
Cancer Treat Res 2019;176:145-161. doi: 10.1007/978-3-319-99716-2_7. PMID: 30596217
Oliveira PD, Farre L, Bittencourt AL
Rev Assoc Med Bras (1992) 2016 Oct;62(7):691-700. doi: 10.1590/1806-9282.62.07.691. PMID: 27925051
Qayyum S, Choi JK
Arch Pathol Lab Med 2014 Feb;138(2):282-6. doi: 10.5858/arpa.2012-0379-RS. PMID: 24476526

Therapy

Katsuya H
Int J Hematol 2023 Apr;117(4):512-522. Epub 2023 Mar 2 doi: 10.1007/s12185-023-03572-4. PMID: 36862273
Ishitsuka K
Semin Hematol 2021 Apr;58(2):114-122. Epub 2021 Mar 3 doi: 10.1053/j.seminhematol.2021.02.005. PMID: 33906721
Mehta-Shah N, Ratner L, Horwitz SM
J Oncol Pract 2017 Aug;13(8):487-492. doi: 10.1200/JOP.2017.021907. PMID: 28796966Free PMC Article
Tsukasaki K
Hematology 2012 Apr;17 Suppl 1:S32-5. doi: 10.1179/102453312X13336169155330. PMID: 22507774
Matutes E
J Clin Pathol 2007 Dec;60(12):1373-7. doi: 10.1136/jcp.2007.052456. PMID: 18042693Free PMC Article

Prognosis

Kogure Y, Kameda T, Koya J, Yoshimitsu M, Nosaka K, Yasunaga JI, Imaizumi Y, Watanabe M, Saito Y, Ito Y, McClure MB, Tabata M, Shingaki S, Yoshifuji K, Chiba K, Okada A, Kakiuchi N, Nannya Y, Kamiunten A, Tahira Y, Akizuki K, Sekine M, Shide K, Hidaka T, Kubuki Y, Kitanaka A, Hidaka M, Nakano N, Utsunomiya A, Sica RA, Acuna-Villaorduna A, Janakiram M, Shah U, Ramos JC, Shibata T, Takeuchi K, Takaori-Kondo A, Miyazaki Y, Matsuoka M, Ishitsuka K, Shiraishi Y, Miyano S, Ogawa S, Ye BH, Shimoda K, Kataoka K
Blood 2022 Feb 17;139(7):967-982. doi: 10.1182/blood.2021013568. PMID: 34695199Free PMC Article
Munakata W, Tobinai K
Cancer Treat Res 2019;176:145-161. doi: 10.1007/978-3-319-99716-2_7. PMID: 30596217
Mehta-Shah N, Ratner L, Horwitz SM
J Oncol Pract 2017 Aug;13(8):487-492. doi: 10.1200/JOP.2017.021907. PMID: 28796966Free PMC Article
Oliveira PD, Farre L, Bittencourt AL
Rev Assoc Med Bras (1992) 2016 Oct;62(7):691-700. doi: 10.1590/1806-9282.62.07.691. PMID: 27925051
Qayyum S, Choi JK
Arch Pathol Lab Med 2014 Feb;138(2):282-6. doi: 10.5858/arpa.2012-0379-RS. PMID: 24476526

Clinical prediction guides

Ohmoto A, Fuji S
Expert Rev Hematol 2023 Jul-Dec;16(7):535-541. Epub 2023 May 18 doi: 10.1080/17474086.2023.2215424. PMID: 37191476
Wolf S, Vercruyssen M, Cook L
Curr Opin Oncol 2022 Sep 1;34(5):446-453. Epub 2022 Jul 25 doi: 10.1097/CCO.0000000000000883. PMID: 35880453
Ishitsuka K
Semin Hematol 2021 Apr;58(2):114-122. Epub 2021 Mar 3 doi: 10.1053/j.seminhematol.2021.02.005. PMID: 33906721
Miyashiro D, Sanches JA
Semin Diagn Pathol 2020 Mar;37(2):81-91. Epub 2019 Jul 30 doi: 10.1053/j.semdp.2019.07.010. PMID: 31387753
Ohshima K
Oncology 2015;89 Suppl 1:7-15. Epub 2015 Nov 10 doi: 10.1159/000431058. PMID: 26550829

Recent systematic reviews

Ohmoto A, Fuji S
Expert Rev Anti Infect Ther 2024 May;22(5):307-316. Epub 2024 Apr 1 doi: 10.1080/14787210.2024.2336547. PMID: 38536666
Shafiee A, Seighali N, Taherzadeh-Ghahfarokhi N, Mardi S, Shojaeian S, Shadabi S, Hasani M, Haghi S, Mozhgani SH
Virol J 2023 Jun 7;20(1):118. doi: 10.1186/s12985-023-02077-0. PMID: 37287047Free PMC Article
Nosaka K, Crawford B, Yi J, Kuan W, Matsumoto T, Takahashi T
Eur J Haematol 2022 Mar;108(3):212-222. Epub 2021 Dec 8 doi: 10.1111/ejh.13728. PMID: 34862665Free PMC Article
Oliveira PD, Kachimarek AC, Bittencourt AL
J Trop Pediatr 2018 Apr 1;64(2):151-161. doi: 10.1093/tropej/fmx039. PMID: 28582585
Oliveira PD, de Carvalho RF, Bittencourt AL
Int J STD AIDS 2017 Mar;28(3):217-228. Epub 2016 Dec 14 doi: 10.1177/0956462416684461. PMID: 28178905

Supplemental Content

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    Clinical resources

    Practice guidelines

    • PubMed
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), T-Cell Lymphomas, 2024

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