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T-cell acute lymphoblastic leukemias

MedGen UID:
368378
Concept ID:
C1961099
Neoplastic Process
Synonyms: Susceptibility to T-cell acute lymphoblastic leukemia; T-cell acute lymphoblastic leukemia; 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; MONDO:0003540

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

López-Nieva P, González-Sánchez L, Cobos-Fernández MÁ, Córdoba R, Santos J, Fernández-Piqueras J
Oncologist 2021 Feb;26(2):e298-e305. Epub 2020 Nov 26 doi: 10.1002/onco.13595. PMID: 33191568Free PMC Article
Schoenaker MH, Suarez F, Szczepanski T, Mahlaoui N, Loeffen JL
Eur J Med Genet 2016 Dec;59(12):641-646. Epub 2016 May 27 doi: 10.1016/j.ejmg.2016.05.012. PMID: 27238889

Curated

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

Recent clinical studies

Etiology

Steimlé T, Dourthe ME, Alcantara M, Touzart A, Simonin M, Mondesir J, Lhermitte L, Bond J, Graux C, Grardel N, Cayuela JM, Arnoux I, Gandemer V, Balsat M, Vey N, Macintyre E, Ifrah N, Dombret H, Petit A, Baruchel A, Ruminy P, Boissel N, Asnafi V
Blood Cancer J 2022 Jan 26;12(1):14. doi: 10.1038/s41408-022-00613-9. PMID: 35082269Free PMC Article
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article
Kim R, Boissel N, Touzart A, Leguay T, Thonier F, Thomas X, Raffoux E, Huguet F, Villarese P, Fourrage C, Passini L, Hunault M, Lepretre S, Chevallier P, Braun T, Lhéritier V, Chantepie S, Maury S, Escoffre M, Tavernier E, Chalandon Y, Graux C, Macintyre E, Ifrah N, Asnafi V, Dombret H, Lhermitte L; on behalf the GRAALL group
Leukemia 2020 Jul;34(7):1730-1740. Epub 2020 Jan 28 doi: 10.1038/s41375-019-0685-4. PMID: 31992840
Meyer LK, Huang BJ, Delgado-Martin C, Roy RP, Hechmer A, Wandler AM, Vincent TL, Fortina P, Olshen AB, Wood BL, Horton TM, Shannon KM, Teachey DT, Hermiston ML
J Clin Invest 2020 Feb 3;130(2):863-876. doi: 10.1172/JCI130189. PMID: 31687977Free PMC Article
Van Vlierberghe P, Ferrando A
J Clin Invest 2012 Oct;122(10):3398-406. Epub 2012 Oct 1 doi: 10.1172/JCI61269. PMID: 23023710Free PMC Article

Diagnosis

Steimlé T, Dourthe ME, Alcantara M, Touzart A, Simonin M, Mondesir J, Lhermitte L, Bond J, Graux C, Grardel N, Cayuela JM, Arnoux I, Gandemer V, Balsat M, Vey N, Macintyre E, Ifrah N, Dombret H, Petit A, Baruchel A, Ruminy P, Boissel N, Asnafi V
Blood Cancer J 2022 Jan 26;12(1):14. doi: 10.1038/s41408-022-00613-9. PMID: 35082269Free PMC Article
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
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article
Meyer LK, Huang BJ, Delgado-Martin C, Roy RP, Hechmer A, Wandler AM, Vincent TL, Fortina P, Olshen AB, Wood BL, Horton TM, Shannon KM, Teachey DT, Hermiston ML
J Clin Invest 2020 Feb 3;130(2):863-876. doi: 10.1172/JCI130189. PMID: 31687977Free PMC Article
Richter-Pechańska P, Kunz JB, Bornhauser B, von Knebel Doeberitz C, Rausch T, Erarslan-Uysal B, Assenov Y, Frismantas V, Marovca B, Waszak SM, Zimmermann M, Seemann J, Happich M, Stanulla M, Schrappe M, Cario G, Escherich G, Bakharevich K, Kirschner-Schwabe R, Eckert C, Muckenthaler MU, Korbel JO, Bourquin JP, Kulozik AE
EMBO Mol Med 2018 Dec;10(12) doi: 10.15252/emmm.201809443. PMID: 30389682Free PMC Article

Therapy

Barz MJ, Behrmann L, Capron D, Zuchtriegel G, Steffen FD, Kunz L, Zhang Y, Vermeerbergen IJ, Marovca B, Kirschmann M, Zech A, Nombela-Arrieta C, Ziegler U, Schroeder T, Bornhauser B, Bourquin JP
Haematologica 2023 May 1;108(5):1244-1258. doi: 10.3324/haematol.2021.280451. PMID: 36325888Free PMC Article
Di Giacomo D, La Starza R, Gorello P, Pellanera F, Kalender Atak Z, De Keersmaecker K, Pierini V, Harrison CJ, Arniani S, Moretti M, Testoni N, De Santis G, Roti G, Matteucci C, Bassan R, Vandenberghe P, Aerts S, Cools J, Bornhauser B, Bourquin JP, Piazza R, Mecucci C
Blood 2021 Sep 2;138(9):773-784. doi: 10.1182/blood.2020010510. PMID: 33876209Free PMC Article
Kim R, Boissel N, Touzart A, Leguay T, Thonier F, Thomas X, Raffoux E, Huguet F, Villarese P, Fourrage C, Passini L, Hunault M, Lepretre S, Chevallier P, Braun T, Lhéritier V, Chantepie S, Maury S, Escoffre M, Tavernier E, Chalandon Y, Graux C, Macintyre E, Ifrah N, Asnafi V, Dombret H, Lhermitte L; on behalf the GRAALL group
Leukemia 2020 Jul;34(7):1730-1740. Epub 2020 Jan 28 doi: 10.1038/s41375-019-0685-4. PMID: 31992840
Meyer LK, Huang BJ, Delgado-Martin C, Roy RP, Hechmer A, Wandler AM, Vincent TL, Fortina P, Olshen AB, Wood BL, Horton TM, Shannon KM, Teachey DT, Hermiston ML
J Clin Invest 2020 Feb 3;130(2):863-876. doi: 10.1172/JCI130189. PMID: 31687977Free PMC Article
Ferrando AA
Hematology Am Soc Hematol Educ Program 2009:353-61. doi: 10.1182/asheducation-2009.1.353. PMID: 20008221Free PMC Article

Prognosis

Barz MJ, Behrmann L, Capron D, Zuchtriegel G, Steffen FD, Kunz L, Zhang Y, Vermeerbergen IJ, Marovca B, Kirschmann M, Zech A, Nombela-Arrieta C, Ziegler U, Schroeder T, Bornhauser B, Bourquin JP
Haematologica 2023 May 1;108(5):1244-1258. doi: 10.3324/haematol.2021.280451. PMID: 36325888Free PMC Article
Steimlé T, Dourthe ME, Alcantara M, Touzart A, Simonin M, Mondesir J, Lhermitte L, Bond J, Graux C, Grardel N, Cayuela JM, Arnoux I, Gandemer V, Balsat M, Vey N, Macintyre E, Ifrah N, Dombret H, Petit A, Baruchel A, Ruminy P, Boissel N, Asnafi V
Blood Cancer J 2022 Jan 26;12(1):14. doi: 10.1038/s41408-022-00613-9. PMID: 35082269Free PMC Article
Meyer LK, Huang BJ, Delgado-Martin C, Roy RP, Hechmer A, Wandler AM, Vincent TL, Fortina P, Olshen AB, Wood BL, Horton TM, Shannon KM, Teachey DT, Hermiston ML
J Clin Invest 2020 Feb 3;130(2):863-876. doi: 10.1172/JCI130189. PMID: 31687977Free PMC Article
La Starza R, Borga C, Barba G, Pierini V, Schwab C, Matteucci C, Lema Fernandez AG, Leszl A, Cazzaniga G, Chiaretti S, Basso G, Harrison CJ, Te Kronnie G, Mecucci C
Blood 2014 Dec 4;124(24):3577-82. Epub 2014 Sep 30 doi: 10.1182/blood-2014-06-578856. PMID: 25270907
Van Vlierberghe P, Ferrando A
J Clin Invest 2012 Oct;122(10):3398-406. Epub 2012 Oct 1 doi: 10.1172/JCI61269. PMID: 23023710Free PMC Article

Clinical prediction guides

Barz MJ, Behrmann L, Capron D, Zuchtriegel G, Steffen FD, Kunz L, Zhang Y, Vermeerbergen IJ, Marovca B, Kirschmann M, Zech A, Nombela-Arrieta C, Ziegler U, Schroeder T, Bornhauser B, Bourquin JP
Haematologica 2023 May 1;108(5):1244-1258. doi: 10.3324/haematol.2021.280451. PMID: 36325888Free PMC Article
López-Nieva P, González-Sánchez L, Cobos-Fernández MÁ, Córdoba R, Santos J, Fernández-Piqueras J
Oncologist 2021 Feb;26(2):e298-e305. Epub 2020 Nov 26 doi: 10.1002/onco.13595. PMID: 33191568Free PMC Article
Bonaccorso P, Bugarin C, Buracchi C, Fazio G, Biondi A, Lo Nigro L, Gaipa G
Cytometry B Clin Cytom 2020 Nov;98(6):491-503. Epub 2020 Jun 1 doi: 10.1002/cyto.b.21882. PMID: 32479694
Richter-Pechańska P, Kunz JB, Bornhauser B, von Knebel Doeberitz C, Rausch T, Erarslan-Uysal B, Assenov Y, Frismantas V, Marovca B, Waszak SM, Zimmermann M, Seemann J, Happich M, Stanulla M, Schrappe M, Cario G, Escherich G, Bakharevich K, Kirschner-Schwabe R, Eckert C, Muckenthaler MU, Korbel JO, Bourquin JP, Kulozik AE
EMBO Mol Med 2018 Dec;10(12) doi: 10.15252/emmm.201809443. PMID: 30389682Free PMC Article
Zhou M, Zhou K, Cheng L, Chen X, Wang J, Wang XM, Zhang Y, Yu Q, Zhang S, Wang D, Huang L, Huang M, Ma D, Cheng T, Wang CY, Yuan W, Zhou J
Cancer Res 2018 Apr 1;78(7):1632-1642. Epub 2018 Jan 12 doi: 10.1158/0008-5472.CAN-17-1434. PMID: 29330145

Supplemental Content

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    Curated

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

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