U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Precursor T-cell acute lymphoblastic leukemia

MedGen UID:
976286
•Concept ID:
CN294276
•
Disease or Syndrome
Synonyms: precursor T-cell acute lymphoblastic leukemia; Precursor T-cell acute lymphoblastic leukemia/lymphoma; precursor T-cell acute lymphoblastic leukemia/lymphoma; precursor T-cell acute lymphocytic leukaemia; Precursor T-cell acute lymphocytic leukemia; precursor T-cell acute lymphocytic leukemia; Precursor T-cell acute lymphocytic leukemia/lymphoma; precursor T-cell acute lymphocytic leukemia/lymphoma; T-ALL
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0020512
Orphanet: ORPHA99861

Definition

A rare acute lymphoblastic leukemia characterized by a neoplasm of lymphoblasts committed to the T-cell lineage, involving bone marrow and blood. A value of >25% bone marrow blasts may be used to define leukemia (as opposed to lymphoma) in cases with the presence of a mass lesion in addition to bone marrow involvement. Patients typically present with leukocytosis, and frequently with a large mediastinal or other tissue mass. Lymphadenopathy and hepatosplenomegaly are common. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPrecursor T-cell acute lymphoblastic leukemia

Professional guidelines

PubMed

Integrated clinical genotype-phenotype characteristics of early T-cell precursor acute lymphoblastic leukemia.
Ye MT, Wang Y, Zuo Z, Calin S, He H, Tang Z, Jabbour EJ, Borthakur G, Zhang Y, Yang Y, You MJ
Cancer 2023 Jan 1;129(1):49-59. Epub 2022 Oct 25 doi: 10.1002/cncr.34515. PMID: 36281717
"Root"ing for successful T-ALL treatment.
Padi SKR, Kraft AS
Blood 2021 May 6;137(18):2422-2423. doi: 10.1182/blood.2020009748. PMID: 33956068Free PMC Article
Recent Advances in the Biology and Treatment of T Cell Acute Lymphoblastic Leukemia.
Hefazi M, Litzow MR
Curr Hematol Malig Rep 2018 Aug;13(4):265-274. doi: 10.1007/s11899-018-0455-9. PMID: 29948644

Recent clinical studies

Etiology

NOTCH1 activation clinically antagonizes the unfavorable effect of PTEN inactivation in BFM-treated children with precursor T-cell acute lymphoblastic leukemia.
Bandapalli OR, Zimmermann M, Kox C, Stanulla M, Schrappe M, Ludwig WD, Koehler R, Muckenthaler MU, Kulozik AE
Haematologica 2013 Jun;98(6):928-36. Epub 2013 Jan 24 doi: 10.3324/haematol.2012.073585. PMID: 23349303Free PMC Article
The favorable effect of activating NOTCH1 receptor mutations on long-term outcome in T-ALL patients treated on the ALL-BFM 2000 protocol can be separated from FBXW7 loss of function.
Kox C, Zimmermann M, Stanulla M, Leible S, Schrappe M, Ludwig WD, Koehler R, Tolle G, Bandapalli OR, Breit S, Muckenthaler MU, Kulozik AE
Leukemia 2010 Dec;24(12):2005-13. Epub 2010 Oct 14 doi: 10.1038/leu.2010.203. PMID: 20944675Free PMC Article
High-resolution genomic profiling of childhood T-ALL reveals frequent copy-number alterations affecting the TGF-beta and PI3K-AKT pathways and deletions at 6q15-16.1 as a genomic marker for unfavorable early treatment response.
Remke M, Pfister S, Kox C, Toedt G, Becker N, Benner A, Werft W, Breit S, Liu S, Engel F, Wittmann A, Zimmermann M, Stanulla M, Schrappe M, Ludwig WD, Bartram CR, Radlwimmer B, Muckenthaler MU, Lichter P, Kulozik AE
Blood 2009 Jul 30;114(5):1053-62. Epub 2009 Apr 30 doi: 10.1182/blood-2008-10-186536. PMID: 19406988
Flow cytometry in the differential diagnosis of lymphocyte-rich thymoma from precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma.
Li S, Juco J, Mann KP, Holden JT
Am J Clin Pathol 2004 Feb;121(2):268-74. doi: 10.1309/K2FY-1TED-8GEG-FLNG. PMID: 14983942
Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets.
Onciu M, Lai R, Vega F, Bueso-Ramos C, Medeiros LJ
Am J Clin Pathol 2002 Feb;117(2):252-8. doi: 10.1309/08DJ-GPBH-H0VR-RC6F. PMID: 11863221

Diagnosis

Pediatric T-cell lymphoblastic leukemia evolves into relapse by clonal selection, acquisition of mutations and promoter hypomethylation.
Kunz JB, Rausch T, Bandapalli OR, Eilers J, Pechanska P, Schuessele S, Assenov Y, Stütz AM, Kirschner-Schwabe R, Hof J, Eckert C, von Stackelberg A, Schrappe M, Stanulla M, Koehler R, Avigad S, Elitzur S, Handgretinger R, Benes V, Weischenfeldt J, Korbel JO, Muckenthaler MU, Kulozik AE
Haematologica 2015 Nov;100(11):1442-50. Epub 2015 Aug 20 doi: 10.3324/haematol.2015.129692. PMID: 26294725Free PMC Article
NOTCH1 activation clinically antagonizes the unfavorable effect of PTEN inactivation in BFM-treated children with precursor T-cell acute lymphoblastic leukemia.
Bandapalli OR, Zimmermann M, Kox C, Stanulla M, Schrappe M, Ludwig WD, Koehler R, Muckenthaler MU, Kulozik AE
Haematologica 2013 Jun;98(6):928-36. Epub 2013 Jan 24 doi: 10.3324/haematol.2012.073585. PMID: 23349303Free PMC Article
Precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma and acute biphenotypic leukemias.
Han X, Bueso-Ramos CE
Am J Clin Pathol 2007 Apr;127(4):528-44. doi: 10.1309/2QE3A6EKQ8UYDYRC. PMID: 17369128
Flow cytometry in the differential diagnosis of lymphocyte-rich thymoma from precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma.
Li S, Juco J, Mann KP, Holden JT
Am J Clin Pathol 2004 Feb;121(2):268-74. doi: 10.1309/K2FY-1TED-8GEG-FLNG. PMID: 14983942
Inhibition of in vitro spontaneous apoptosis by IL-7 correlates with bcl-2 up-regulation, cortical/mature immunophenotype, and better early cytoreduction of childhood T-cell acute lymphoblastic leukemia.
Karawajew L, Ruppert V, Wuchter C, Kösser A, Schrappe M, Dörken B, Ludwig WD
Blood 2000 Jul 1;96(1):297-306. PMID: 10891465

Therapy

Pediatric T-cell lymphoblastic leukemia evolves into relapse by clonal selection, acquisition of mutations and promoter hypomethylation.
Kunz JB, Rausch T, Bandapalli OR, Eilers J, Pechanska P, Schuessele S, Assenov Y, Stütz AM, Kirschner-Schwabe R, Hof J, Eckert C, von Stackelberg A, Schrappe M, Stanulla M, Koehler R, Avigad S, Elitzur S, Handgretinger R, Benes V, Weischenfeldt J, Korbel JO, Muckenthaler MU, Kulozik AE
Haematologica 2015 Nov;100(11):1442-50. Epub 2015 Aug 20 doi: 10.3324/haematol.2015.129692. PMID: 26294725Free PMC Article
The favorable effect of activating NOTCH1 receptor mutations on long-term outcome in T-ALL patients treated on the ALL-BFM 2000 protocol can be separated from FBXW7 loss of function.
Kox C, Zimmermann M, Stanulla M, Leible S, Schrappe M, Ludwig WD, Koehler R, Tolle G, Bandapalli OR, Breit S, Muckenthaler MU, Kulozik AE
Leukemia 2010 Dec;24(12):2005-13. Epub 2010 Oct 14 doi: 10.1038/leu.2010.203. PMID: 20944675Free PMC Article
High-resolution genomic profiling of childhood T-ALL reveals frequent copy-number alterations affecting the TGF-beta and PI3K-AKT pathways and deletions at 6q15-16.1 as a genomic marker for unfavorable early treatment response.
Remke M, Pfister S, Kox C, Toedt G, Becker N, Benner A, Werft W, Breit S, Liu S, Engel F, Wittmann A, Zimmermann M, Stanulla M, Schrappe M, Ludwig WD, Bartram CR, Radlwimmer B, Muckenthaler MU, Lichter P, Kulozik AE
Blood 2009 Jul 30;114(5):1053-62. Epub 2009 Apr 30 doi: 10.1182/blood-2008-10-186536. PMID: 19406988
A case of chronic myeloproliferative syndrome followed by precursor T-cell acute lymphoblastic leukemia.
Bacher U, Haferlach T, Kern W, Harich HD, Schnittger S, Haferlach C
Cancer Genet Cytogenet 2007 May;175(1):52-6. doi: 10.1016/j.cancergencyto.2006.12.007. PMID: 17498558
Heterogeneous CD52 expression among hematologic neoplasms: implications for the use of alemtuzumab (CAMPATH-1H).
Rodig SJ, Abramson JS, Pinkus GS, Treon SP, Dorfman DM, Dong HY, Shipp MA, Kutok JL
Clin Cancer Res 2006 Dec 1;12(23):7174-9. doi: 10.1158/1078-0432.CCR-06-1275. PMID: 17145843

Prognosis

NOTCH1 activation clinically antagonizes the unfavorable effect of PTEN inactivation in BFM-treated children with precursor T-cell acute lymphoblastic leukemia.
Bandapalli OR, Zimmermann M, Kox C, Stanulla M, Schrappe M, Ludwig WD, Koehler R, Muckenthaler MU, Kulozik AE
Haematologica 2013 Jun;98(6):928-36. Epub 2013 Jan 24 doi: 10.3324/haematol.2012.073585. PMID: 23349303Free PMC Article
Aberrant myeloid marker expression in precursor B-cell and T-cell leukemias.
Suggs JL, Cruse JM, Lewis RE
Exp Mol Pathol 2007 Dec;83(3):471-3. Epub 2007 Sep 7 doi: 10.1016/j.yexmp.2007.08.012. PMID: 17963747
Precursor T-cell acute lymphoblastic leukemia/lymphoblastic lymphoma and acute biphenotypic leukemias.
Han X, Bueso-Ramos CE
Am J Clin Pathol 2007 Apr;127(4):528-44. doi: 10.1309/2QE3A6EKQ8UYDYRC. PMID: 17369128
Precursor T-cell acute lymphoblastic leukemia/lymphoma involving the uterine cervix, myometrium, endometrium, and appendix.
Lyman MD, Neuhauser TS
Ann Diagn Pathol 2002 Apr;6(2):125-8. doi: 10.1053/adpa.2002.32381. PMID: 12004362
Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets.
Onciu M, Lai R, Vega F, Bueso-Ramos C, Medeiros LJ
Am J Clin Pathol 2002 Feb;117(2):252-8. doi: 10.1309/08DJ-GPBH-H0VR-RC6F. PMID: 11863221

Clinical prediction guides

A Significant Influence of Metronidazole on Busulfan Pharmacokinetics: A Case Report of Therapeutic Drug Monitoring.
Chung H, Yu KS, Hong KT, Choi JY, Hong CR, Kang HJ, Park KD, Shin HY, Lee S
Ther Drug Monit 2017 Jun;39(3):208-210. doi: 10.1097/FTD.0000000000000395. PMID: 28328762
Loss of RASSF1A synergizes with deregulated RUNX2 signaling in tumorigenesis.
van der Weyden L, Papaspyropoulos A, Poulogiannis G, Rust AG, Rashid M, Adams DJ, Arends MJ, O'Neill E
Cancer Res 2012 Aug 1;72(15):3817-3827. Epub 2012 Jun 18 doi: 10.1158/0008-5472.CAN-11-3343. PMID: 22710434Free PMC Article
The favorable effect of activating NOTCH1 receptor mutations on long-term outcome in T-ALL patients treated on the ALL-BFM 2000 protocol can be separated from FBXW7 loss of function.
Kox C, Zimmermann M, Stanulla M, Leible S, Schrappe M, Ludwig WD, Koehler R, Tolle G, Bandapalli OR, Breit S, Muckenthaler MU, Kulozik AE
Leukemia 2010 Dec;24(12):2005-13. Epub 2010 Oct 14 doi: 10.1038/leu.2010.203. PMID: 20944675Free PMC Article
High-resolution genomic profiling of childhood T-ALL reveals frequent copy-number alterations affecting the TGF-beta and PI3K-AKT pathways and deletions at 6q15-16.1 as a genomic marker for unfavorable early treatment response.
Remke M, Pfister S, Kox C, Toedt G, Becker N, Benner A, Werft W, Breit S, Liu S, Engel F, Wittmann A, Zimmermann M, Stanulla M, Schrappe M, Ludwig WD, Bartram CR, Radlwimmer B, Muckenthaler MU, Lichter P, Kulozik AE
Blood 2009 Jul 30;114(5):1053-62. Epub 2009 Apr 30 doi: 10.1182/blood-2008-10-186536. PMID: 19406988
Precursor T-cell acute lymphoblastic leukemia in adults: age-related immunophenotypic, cytogenetic, and molecular subsets.
Onciu M, Lai R, Vega F, Bueso-Ramos C, Medeiros LJ
Am J Clin Pathol 2002 Feb;117(2):252-8. doi: 10.1309/08DJ-GPBH-H0VR-RC6F. PMID: 11863221

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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

    Reviews

    Related information

    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...