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T-cell acute lymphoblastic leukemia

MedGen UID:
368378
Concept ID:
C1961099
Neoplastic Process
Synonyms: Susceptibility to T-cell acute lymphoblastic leukemia; T-cell acute lymphoblastic leukemias; T-cell acute lymphocytic leukemia, somatic
SNOMED CT: Pro-T ALL (128824003); Pre-T ALL (128824003); Cortical T ALL (128824003); Precursor T-cell lymphoblastic leukemia (128824003); Precursor T-cell lymphoblastic leukemia (277575008); Acute T-cell lymphoblastic leukemia (277575008); T-cell acute lymphoblastic leukemia (277575008)
 
HPO: HP:0006727
Monarch Initiative: MONDO:0004963

Definition

Acute lymphoblastic leukemia of T-cell origin. It comprises about 15% of childhood cases and 25% of adult cases. It is more common in males than females. [from HPO]

Conditions with this feature

Fanconi anemia complementation group D1
MedGen UID:
325420
Concept ID:
C1838457
Disease or Syndrome
Fanconi anemia (FA) is characterized by physical abnormalities, bone marrow failure, and increased risk for malignancy. Physical abnormalities, present in approximately 75% of affected individuals, include one or more of the following: short stature, abnormal skin pigmentation, skeletal malformations of the upper and/or lower limbs, microcephaly, and ophthalmic and genitourinary tract anomalies. Progressive bone marrow failure with pancytopenia typically presents in the first decade, often initially with thrombocytopenia or leukopenia. The incidence of acute myeloid leukemia is 13% by age 50 years. Solid tumors – particularly of the head and neck, skin, and genitourinary tract – are more common in individuals with FA.
Lymphoblastic leukemia, acute, with lymphomatous features
MedGen UID:
340879
Concept ID:
C1855472
Neoplastic Process
Glioma susceptibility 3
MedGen UID:
442777
Concept ID:
C2751641
Finding
Any malignant glioma in which the cause of the disease is a mutation in the BRCA2 gene.
Mismatch repair cancer syndrome 2
MedGen UID:
1750327
Concept ID:
C5436806
Disease or Syndrome
Mismatch repair cancer syndrome-2 (MMRCS2) is an autosomal recessive childhood cancer predisposition syndrome characterized by hematologic malignancy, brain tumors, and gastrointestinal tumors. Multiple cafe-au-lait spots reminiscent of neurofibromatosis type I (NF1; 162200) may be present. Microsatellite instability may be detected in tumor samples (Muller et al., 2006). For a discussion of genetic heterogeneity of mismatch repair cancer syndrome (MMRCS), see MMRCS1 (276300).
Bone marrow failure and diabetes mellitus syndrome
MedGen UID:
1823991
Concept ID:
C5774218
Disease or Syndrome
Bone marrow failure and diabetes mellitus syndrome (BMFDMS) is an autosomal recessive disorder characterized by the onset of manifestations of bone marrow failure, such as anemia, thrombocytopenia, and dyserythropoiesis, in infancy or early childhood. White blood cell lineages may or may not be affected. Patients with BMFDMS also develop nonautoimmune insulin-dependent diabetes mellitus in the first or second decades, likely due to apoptosis of pancreatic beta cells. Many patients show pigmentary skin abnormalities and short stature. Bone marrow transplant is curative for the bone marrow failure, but does not have an effect on diabetes (Dos Santos et al., 2017).

Professional guidelines

PubMed

Leoncin M, La Starza R, Roti G, Pagliaro L, Bassan R, Mecucci C
Curr Opin Oncol 2022 Nov 1;34(6):738-747. Epub 2022 Aug 26 doi: 10.1097/CCO.0000000000000900. PMID: 36017547
Cooper ML, Choi J, Staser K, Ritchey JK, Devenport JM, Eckardt K, Rettig MP, Wang B, Eissenberg LG, Ghobadi A, Gehrs LN, Prior JL, Achilefu S, Miller CA, Fronick CC, O'Neal J, Gao F, Weinstock DM, Gutierrez A, Fulton RS, DiPersio JF
Leukemia 2018 Sep;32(9):1970-1983. Epub 2018 Feb 20 doi: 10.1038/s41375-018-0065-5. PMID: 29483708Free PMC Article
Marks DI, Rowntree C
Blood 2017 Mar 2;129(9):1134-1142. Epub 2017 Jan 23 doi: 10.1182/blood-2016-07-692608. PMID: 28115371

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Acute Lymphoblastic Leukemia, 2023

Recent clinical studies

Etiology

Chiesa R, Georgiadis C, Syed F, Zhan H, Etuk A, Gkazi SA, Preece R, Ottaviano G, Braybrook T, Chu J, Kubat A, Adams S, Thomas R, Gilmour K, O'Connor D, Vora A, Qasim W; Base-Edited CAR T Group
N Engl J Med 2023 Sep 7;389(10):899-910. Epub 2023 Jun 14 doi: 10.1056/NEJMoa2300709. PMID: 37314354
Teachey DT, Devidas M, Wood BL, Chen Z, Hayashi RJ, Hermiston ML, Annett RD, Archer JH, Asselin BL, August KJ, Cho SY, Dunsmore KP, Fisher BT, Freedman JL, Galardy PJ, Harker-Murray P, Horton TM, Jaju AI, Lam A, Messinger YH, Miles RR, Okada M, Patel SI, Schafer ES, Schechter T, Singh N, Steele AC, Sulis ML, Vargas SL, Winter SS, Wood C, Zweidler-McKay P, Bollard CM, Loh ML, Hunger SP, Raetz EA
J Clin Oncol 2022 Jul 1;40(19):2106-2118. Epub 2022 Mar 10 doi: 10.1200/JCO.21.02678. PMID: 35271306Free PMC Article
Dunsmore KP, Winter SS, Devidas M, Wood BL, Esiashvili N, Chen Z, Eisenberg N, Briegel N, Hayashi RJ, Gastier-Foster JM, Carroll AJ, Heerema NA, Asselin BL, Rabin KR, Zweidler-Mckay PA, Raetz EA, Loh ML, Schultz KR, Winick NJ, Carroll WL, Hunger SP
J Clin Oncol 2020 Oct 1;38(28):3282-3293. Epub 2020 Aug 19 doi: 10.1200/JCO.20.00256. PMID: 32813610Free PMC Article
Vadillo E, Dorantes-Acosta E, Pelayo R, Schnoor M
Blood Rev 2018 Jan;32(1):36-51. Epub 2017 Aug 15 doi: 10.1016/j.blre.2017.08.006. PMID: 28830639
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Diagnosis

Si Lim SJ, Ford JB, Hermiston ML
Blood 2023 Jun 22;141(25):3019-3030. doi: 10.1182/blood.2022016503. PMID: 37018730
Bardelli V, Arniani S, Pierini V, Di Giacomo D, Pierini T, Gorello P, Mecucci C, La Starza R
Genes (Basel) 2021 Jul 23;12(8) doi: 10.3390/genes12081118. PMID: 34440292Free PMC Article
Dunsmore KP, Winter SS, Devidas M, Wood BL, Esiashvili N, Chen Z, Eisenberg N, Briegel N, Hayashi RJ, Gastier-Foster JM, Carroll AJ, Heerema NA, Asselin BL, Rabin KR, Zweidler-Mckay PA, Raetz EA, Loh ML, Schultz KR, Winick NJ, Carroll WL, Hunger SP
J Clin Oncol 2020 Oct 1;38(28):3282-3293. Epub 2020 Aug 19 doi: 10.1200/JCO.20.00256. PMID: 32813610Free PMC Article
Kruse A, Abdel-Azim N, Kim HN, Ruan Y, Phan V, Ogana H, Wang W, Lee R, Gang EJ, Khazal S, Kim YM
Int J Mol Sci 2020 Feb 5;21(3) doi: 10.3390/ijms21031054. PMID: 32033444Free PMC Article
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Therapy

Chiesa R, Georgiadis C, Syed F, Zhan H, Etuk A, Gkazi SA, Preece R, Ottaviano G, Braybrook T, Chu J, Kubat A, Adams S, Thomas R, Gilmour K, O'Connor D, Vora A, Qasim W; Base-Edited CAR T Group
N Engl J Med 2023 Sep 7;389(10):899-910. Epub 2023 Jun 14 doi: 10.1056/NEJMoa2300709. PMID: 37314354
Zhang M, Chen D, Fu X, Meng H, Nan F, Sun Z, Yu H, Zhang L, Li L, Li X, Wang X, Wang M, You F, Li Z, Chang Y, Zhou Z, Yan J, Li J, Wu X, Wang Y, Wang Y, Xiang S, Chen Y, Pan G, Xu H, Zhang B, Yang L
Clin Cancer Res 2022 Jul 1;28(13):2830-2843. doi: 10.1158/1078-0432.CCR-21-4097. PMID: 35435984
Teachey DT, Devidas M, Wood BL, Chen Z, Hayashi RJ, Hermiston ML, Annett RD, Archer JH, Asselin BL, August KJ, Cho SY, Dunsmore KP, Fisher BT, Freedman JL, Galardy PJ, Harker-Murray P, Horton TM, Jaju AI, Lam A, Messinger YH, Miles RR, Okada M, Patel SI, Schafer ES, Schechter T, Singh N, Steele AC, Sulis ML, Vargas SL, Winter SS, Wood C, Zweidler-McKay P, Bollard CM, Loh ML, Hunger SP, Raetz EA
J Clin Oncol 2022 Jul 1;40(19):2106-2118. Epub 2022 Mar 10 doi: 10.1200/JCO.21.02678. PMID: 35271306Free PMC Article
Lato MW, Przysucha A, Grosman S, Zawitkowska J, Lejman M
Int J Mol Sci 2021 Apr 26;22(9) doi: 10.3390/ijms22094502. PMID: 33925883Free PMC Article
Dunsmore KP, Winter SS, Devidas M, Wood BL, Esiashvili N, Chen Z, Eisenberg N, Briegel N, Hayashi RJ, Gastier-Foster JM, Carroll AJ, Heerema NA, Asselin BL, Rabin KR, Zweidler-Mckay PA, Raetz EA, Loh ML, Schultz KR, Winick NJ, Carroll WL, Hunger SP
J Clin Oncol 2020 Oct 1;38(28):3282-3293. Epub 2020 Aug 19 doi: 10.1200/JCO.20.00256. PMID: 32813610Free PMC Article

Prognosis

Lato MW, Przysucha A, Grosman S, Zawitkowska J, Lejman M
Int J Mol Sci 2021 Apr 26;22(9) doi: 10.3390/ijms22094502. PMID: 33925883Free PMC Article
Dunsmore KP, Winter SS, Devidas M, Wood BL, Esiashvili N, Chen Z, Eisenberg N, Briegel N, Hayashi RJ, Gastier-Foster JM, Carroll AJ, Heerema NA, Asselin BL, Rabin KR, Zweidler-Mckay PA, Raetz EA, Loh ML, Schultz KR, Winick NJ, Carroll WL, Hunger SP
J Clin Oncol 2020 Oct 1;38(28):3282-3293. Epub 2020 Aug 19 doi: 10.1200/JCO.20.00256. PMID: 32813610Free PMC Article
Kruse A, Abdel-Azim N, Kim HN, Ruan Y, Phan V, Ogana H, Wang W, Lee R, Gang EJ, Khazal S, Kim YM
Int J Mol Sci 2020 Feb 5;21(3) doi: 10.3390/ijms21031054. PMID: 32033444Free PMC Article
McMahon CM, Luger SM
Curr Hematol Malig Rep 2019 Apr;14(2):83-93. doi: 10.1007/s11899-019-00501-3. PMID: 30880359
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Clinical prediction guides

Freiwan A, Zoine JT, Crawford JC, Vaidya A, Schattgen SA, Myers JA, Patil SL, Khanlari M, Inaba H, Klco JM, Mullighan CG, Krenciute G, Chockley PJ, Naik S, Langfitt DM, Mamonkin M, Obeng EA, Thomas PG, Gottschalk S, Velasquez MP
Blood 2022 Dec 22;140(25):2684-2696. doi: 10.1182/blood.2021015020. PMID: 35914226Free PMC Article
Zhang M, Chen D, Fu X, Meng H, Nan F, Sun Z, Yu H, Zhang L, Li L, Li X, Wang X, Wang M, You F, Li Z, Chang Y, Zhou Z, Yan J, Li J, Wu X, Wang Y, Wang Y, Xiang S, Chen Y, Pan G, Xu H, Zhang B, Yang L
Clin Cancer Res 2022 Jul 1;28(13):2830-2843. doi: 10.1158/1078-0432.CCR-21-4097. PMID: 35435984
Kruse A, Abdel-Azim N, Kim HN, Ruan Y, Phan V, Ogana H, Wang W, Lee R, Gang EJ, Khazal S, Kim YM
Int J Mol Sci 2020 Feb 5;21(3) doi: 10.3390/ijms21031054. PMID: 32033444Free PMC Article
Parvin S, Ramirez-Labrada A, Aumann S, Lu X, Weich N, Santiago G, Cortizas EM, Sharabi E, Zhang Y, Sanchez-Garcia I, Gentles AJ, Roberts E, Bilbao-Cortes D, Vega F, Chapman JR, Verdun RE, Lossos IS
Cancer Cell 2019 Sep 16;36(3):237-249.e6. Epub 2019 Aug 22 doi: 10.1016/j.ccell.2019.07.007. PMID: 31447348Free PMC Article
Raetz EA, Teachey DT
Hematology Am Soc Hematol Educ Program 2016 Dec 2;2016(1):580-588. doi: 10.1182/asheducation-2016.1.580. PMID: 27913532Free PMC Article

Recent systematic reviews

Wang J, Zhan QR, Lu XX, Zhang LJ, Wang XX, Zhang HY
Medicine (Baltimore) 2022 Jul 29;101(30):e29294. doi: 10.1097/MD.0000000000029294. PMID: 35905214Free PMC Article
Kathpalia M, Mishra P, Bajpai R, Bhurani D, Agarwal N
Ann Hematol 2022 Aug;101(8):1655-1666. Epub 2022 Jun 21 doi: 10.1007/s00277-022-04880-1. PMID: 35727338
Byun JM, Baek SK, Yoon HJ, Kim SY, Maeng CH, Park TS, Kim HJ, Choi YY, Um YJ
Lab Med 2015 Spring;46(2):140-5. doi: 10.1309/LMU4KMJDRM3AD6FQ. PMID: 25918193

Supplemental Content

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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, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Acute Lymphoblastic Leukemia, 2023

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