Compendium of Cancer Genome Aberrations

HAEM5:Early T-precursor lymphoblastic leukaemia / lymphoma: Difference between revisions

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<blockquote class='blockedit'>{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Early T-Cell Precursor Lymphoblastic Leukemia]].
<blockquote class="blockedit">{{Box-round|title=Content Update To WHO 5th Edition Classification Is In Process; Content Below is Based on WHO 4th Edition Classification|This page was converted to the new template on 2023-12-07. The original page can be found at [[HAEM4:Early T-Cell Precursor Lymphoblastic Leukemia]].
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Fei Yang, MD, FACMG, Kaiser Permanente Northwest
Fei Yang, MD, FACMG, Kaiser Permanente Northwest
__TOC__
==WHO Classification of Disease==
==WHO Classification of Disease==


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==Definition / Description of Disease==
==Related Terminology==
 
Early T-Cell Precursor Lymphoblastic Leukemia (ETP-ALL) is a subtype of T-Lymphoblastic Leukemia (T-ALL) and is suggested to derive from thymic cells at the early T-cell precursor (ETP) differentiation stage<ref name=":10">{{Cite journal|last=Coustan-Smith|first=Elaine|last2=Mullighan|first2=Charles G.|last3=Onciu|first3=Mihaela|last4=Behm|first4=Frederick G.|last5=Raimondi|first5=Susana C.|last6=Pei|first6=Deqing|last7=Cheng|first7=Cheng|last8=Su|first8=Xiaoping|last9=Rubnitz|first9=Jeffrey E.|date=2009-02|title=Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia|url=https://pubmed.ncbi.nlm.nih.gov/19147408|journal=The Lancet. Oncology|volume=10|issue=2|pages=147–156|doi=10.1016/S1470-2045(08)70314-0|issn=1474-5488|pmc=2840241|pmid=19147408}}</ref>. The normal counterpart is the ETP cells that are the earliest thymic progenitors immigrated from the bone marrow to the thymus, with retention of a certain level of multilineage pluripotency rather than common lymphoid progenitors <ref name=":2">{{Cite journal|last=Jain|first=Nitin|last2=Lamb|first2=Audrey V.|last3=O'Brien|first3=Susan|last4=Ravandi|first4=Farhad|last5=Konopleva|first5=Marina|last6=Jabbour|first6=Elias|last7=Zuo|first7=Zhuang|last8=Jorgensen|first8=Jeffrey|last9=Lin|first9=Pei|date=2016-04-14|title=Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype|url=https://pubmed.ncbi.nlm.nih.gov/26747249|journal=Blood|volume=127|issue=15|pages=1863–1869|doi=10.1182/blood-2015-08-661702|issn=1528-0020|pmc=4915808|pmid=26747249}}</ref>. The ETP-ALL subtype has characteristic immunophenotypic and genomic profile compared with other subtypes of T-ALL. Under the 2016 version World Health Organization (WHO) classification<ref name=":0" />, ETP-ALL is defined based on the immunophenotype of the leukemic cells:
 
#positive for intracytoplasmic CD3 and CD7, and positive (≥25% of blasts population) for at least one stem cell/myeloid marker (CD117, CD34, HLA-DR, CD13, CD33, CD11b, or CD65)
#negative to dim positive for CD5 (<75% positive)
#negative for CD1a, CD8, and MPO
 
However, the aforementioned immunophenotypic criteria have been reported not be able to identify all ETP-ALL cases as detected by gene expression profiling, and "negativity for CD4" has been proposed to be added to the criteria <ref>{{Cite journal|last=Zuurbier|first=Linda|last2=Gutierrez|first2=Alejandro|last3=Mullighan|first3=Charles G.|last4=Canté-Barrett|first4=Kirsten|last5=Gevaert|first5=A. Olivier|last6=de Rooi|first6=Johan|last7=Li|first7=Yunlei|last8=Smits|first8=Willem K.|last9=Buijs-Gladdines|first9=Jessica G. C. A. M.|date=2014-01|title=Immature MEF2C-dysregulated T-cell leukemia patients have an early T-cell precursor acute lymphoblastic leukemia gene signature and typically have non-rearranged T-cell receptors|url=https://pubmed.ncbi.nlm.nih.gov/23975177|journal=Haematologica|volume=99|issue=1|pages=94–102|doi=10.3324/haematol.2013.090233|issn=1592-8721|pmc=4007923|pmid=23975177}}</ref>.
 
==Synonyms / Terminology==
 
Early thymic precursor (ETP) acute lymphoblastic leukemia (ALL)<ref>{{Cite journal|last=Bond|first=Jonathan|last2=Graux|first2=Carlos|last3=Lhermitte|first3=Ludovic|last4=Lara|first4=Diane|last5=Cluzeau|first5=Thomas|last6=Leguay|first6=Thibaut|last7=Cieslak|first7=Agata|last8=Trinquand|first8=Amélie|last9=Pastoret|first9=Cedric|date=2017-08-10|title=Early Response-Based Therapy Stratification Improves Survival in Adult Early Thymic Precursor Acute Lymphoblastic Leukemia: A Group for Research on Adult Acute Lymphoblastic Leukemia Study|url=https://pubmed.ncbi.nlm.nih.gov/28605290|journal=Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology|volume=35|issue=23|pages=2683–2691|doi=10.1200/JCO.2016.71.8585|issn=1527-7755|pmid=28605290}}</ref>
 
==Epidemiology / Prevalence==
 
Early T-Cell Precursor Lymphoblastic Leukemia (ETP-ALL) is uncommon, reported in about 5-17% of pediatric acute lymphoblastic leukemia (ALL) and 5-10% of adult ALL cases, respectively<ref name=":2" /><ref name=":5">{{Cite journal|last=Sin|first=Chun-Fung|last2=Man|first2=Pui-Hei Marcus|date=2021|title=Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies|url=https://pubmed.ncbi.nlm.nih.gov/34912707|journal=Frontiers in Oncology|volume=11|pages=750789|doi=10.3389/fonc.2021.750789|issn=2234-943X|pmc=8666570|pmid=34912707}}</ref><ref name=":4" />.
 
==Clinical Features==
 
Put your text here and fill in the table <span style="color:#0070C0">(''Instruction: Can include references in the table. Do not delete table.'') </span>
{| class="wikitable"
|'''Signs and Symptoms'''
|<span class="blue-text">EXAMPLE:</span> Asymptomatic (incidental finding on complete blood counts)
 
<span class="blue-text">EXAMPLE:</span> B-symptoms (weight loss, fever, night sweats)
 
<span class="blue-text">EXAMPLE:</span> Fatigue
 
<span class="blue-text">EXAMPLE:</span> Lymphadenopathy (uncommon)
|-
|'''Laboratory Findings'''
|<span class="blue-text">EXAMPLE:</span> Cytopenias
 
<span class="blue-text">EXAMPLE:</span> Lymphocytosis (low level)
|}
 
 
<blockquote class='blockedit'>{{Box-round|title=v4:Clinical Features|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>
 
The clinical features of ETP-ALL are similar to that of other subtypes of T-ALL, including a high leukocyte count, anterior mediastinal mass or other tissue mass, lymphadenopathy, hepatosplenomegaly<ref name=":3">{{Cite journal|last=Inukai|first=Takeshi|last2=Kiyokawa|first2=Nobutaka|last3=Campana|first3=Dario|last4=Coustan-Smith|first4=Elaine|last5=Kikuchi|first5=Akira|last6=Kobayashi|first6=Miyuki|last7=Takahashi|first7=Hiroyuki|last8=Koh|first8=Katsuyoshi|last9=Manabe|first9=Atsushi|date=2012-02|title=Clinical significance of early T-cell precursor acute lymphoblastic leukaemia: results of the Tokyo Children's Cancer Study Group Study L99-15|url=https://pubmed.ncbi.nlm.nih.gov/22128890|journal=British Journal of Haematology|volume=156|issue=3|pages=358–365|doi=10.1111/j.1365-2141.2011.08955.x|issn=1365-2141|pmid=22128890}}</ref><ref name=":4">Borowitz MJ, et al., (2016).T-lymphoblastic leukemia/lymphoma, in World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4thedition.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J, Vardiman JW, Editors. IARC Press: Lyon, France, p176-178.</ref>. Some patients who develop an anterior mediastinal mass can lead to superior vena cava syndrome.
<blockquote class="blockedit">
<center><span style="color:Maroon">'''End of V4 Section'''</span>
----
</blockquote>
==Sites of Involvement==
 
Similar to non-ETP T-ALL, the sites of involvement include bone marrow, lymph node, extra nodal and anterior mediastinal mass (thymus)<ref name=":3" /><ref name=":4" />.
 
==Morphologic Features==
 
Currently there is no specific morphologic feature reported for ETP-ALL.
 
==Immunophenotype==
 
{| class="wikitable sortable"
|-
!Finding!!Marker
|-
|Positive (universal)||intracytoplasmic CD3, CD7, and at least one stem cell or myeloid antigen (CD34, HLA-DR, CD13, CD33, CD117, CD11b, CD65)<ref name=":0">{{Cite journal|last=Arber|first=Daniel A.|last2=Orazi|first2=Attilio|last3=Hasserjian|first3=Robert|last4=Thiele|first4=Jürgen|last5=Borowitz|first5=Michael J.|last6=Le Beau|first6=Michelle M.|last7=Bloomfield|first7=Clara D.|last8=Cazzola|first8=Mario|last9=Vardiman|first9=James W.|date=2016-05-19|title=The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia|url=https://pubmed.ncbi.nlm.nih.gov/27069254|journal=Blood|volume=127|issue=20|pages=2391–2405|doi=10.1182/blood-2016-03-643544|issn=1528-0020|pmid=27069254}}</ref><ref>{{Cite journal|last=Raetz|first=Elizabeth A.|last2=Teachey|first2=David T.|date=2016-12-02|title=T-cell acute lymphoblastic leukemia|url=https://ashpublications.org/hematology/article/2016/1/580/21136/Tcell-acute-lymphoblastic-leukemia|journal=Hematology|language=en|volume=2016|issue=1|pages=580–588|doi=10.1182/asheducation-2016.1.580|issn=1520-4391|pmc=PMC6142501|pmid=27913532}}</ref><ref name=":5" />
|-
|Positive (subset)||dim expression of CD5 (<75% positive)<ref name=":0" /><ref name=":5" />, CD2<ref name=":0" />
|-
|Negative (universal)||CD1a, CD8<ref name=":0" /><ref name=":5" />
|-
|Negative (subset)||CD5<ref name=":0" /><ref name=":5" />
|}


==WHO Essential and Desirable Genetic Diagnostic Criteria==
<span style="color:#0070C0">(''Instructions: The table will have the diagnostic criteria from the WHO book <u>autocompleted</u>; remove any <u>non</u>-genetics related criteria. If applicable, add text about other classification'' ''systems that define this entity and specify how the genetics-related criteria differ.'')</span>
{| class="wikitable"
|+
|WHO Essential Criteria (Genetics)*
|
|-
|WHO Desirable Criteria (Genetics)*
|
|-
|Other Classification
|
|}
<nowiki>*</nowiki>Note: These are only the genetic/genomic criteria. Additional diagnostic criteria can be found in the [https://tumourclassification.iarc.who.int/home <u>WHO Classification of Tumours</u>].
==Related Terminology==
<span style="color:#0070C0">(''Instructions: The table will have the related terminology from the WHO <u>autocompleted</u>.)''</span>
{| class="wikitable"
{| class="wikitable"
|+
|+
|Acceptable
|Acceptable
|
|N/A
|-
|-
|Not Recommended
|Not Recommended
|
|N/A
|}
|}


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|}


<blockquote class='blockedit'>{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Chromosomal Rearrangements (Gene Fusions)|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>


''MEF2C'' (5q14) rearrangement or rearrangement involving ''MEF2C''-related cofactors have been reported in about 50% of ETP-ALL cases<ref>Homminga I, Pieters R, Langerak A, de Rooi J, Stubbs A, Verstegen M, et al. MEF2C as Novel Oncogene for Early T-Cell Precursor (ETP) Leukemia. ''Blood'' (2010) 116:9–9. doi: 10.1182/blood.V116.21.9.9</ref>, which have been validated in an independent ETP-ALL patient cohort<ref>Meijer M, Cordo V, Hagelaar R, Smits W, Meijerink J. Manipulating MEF2C: Discovering Novel Drugs to Target ETP-ALL. ''Blood'' (2021) 138 (Supplement 1): 3325. doi.org/10.1182/blood-2021-150176.</ref>. Ectopic MEF2C expression due to rearrangement has been demonstrated as an oncogenic driver of ETP-ALL by upregulating ''LMO2'' and ''LYL1,'' which lead to differentiation block of early thymocytes.  
''MEF2C'' (5q14) rearrangement or rearrangement involving ''MEF2C''-related cofactors have been reported in about 50% of ETP-ALL cases<ref>Homminga I, Pieters R, Langerak A, de Rooi J, Stubbs A, Verstegen M, et al. MEF2C as Novel Oncogene for Early T-Cell Precursor (ETP) Leukemia. ''Blood'' (2010) 116:9–9. doi: 10.1182/blood.V116.21.9.9</ref>, which have been validated in an independent ETP-ALL patient cohort<ref>Meijer M, Cordo V, Hagelaar R, Smits W, Meijerink J. Manipulating MEF2C: Discovering Novel Drugs to Target ETP-ALL. ''Blood'' (2021) 138 (Supplement 1): 3325. doi.org/10.1182/blood-2021-150176.</ref>. Ectopic MEF2C expression due to rearrangement has been demonstrated as an oncogenic driver of ETP-ALL by upregulating ''LMO2'' and ''LYL1,'' which lead to differentiation block of early thymocytes.  
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{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
!Chr #!!'''Gain, Loss, Amp, LOH'''!!'''Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]'''!!'''Relevant Gene(s)'''
!Chr #!!Gain, Loss, Amp, LOH!!Minimal Region Cytoband and/or Genomic Coordinates [Genome Build; Size]!!Relevant Gene(s)
!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T'''
!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
!Established Clinical Significance Per Guidelines - Yes or No (Source)
!'''Clinical Relevance Details/Other Notes'''
!Clinical Relevance Details/Other Notes
|-
|-
|<span class="blue-text">EXAMPLE:</span>
|<span class="blue-text">EXAMPLE:</span>
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|}
|}


<blockquote class='blockedit'>{{Box-round|title=v4:Individual Region Genomic Gain/Loss/LOH|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Individual Region Genomic Gain/Loss/LOH|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>


Currently there is no specific copy number alterations/LOH that is associated with ETP-ALL.
Currently there is no specific copy number alterations/LOH that is associated with ETP-ALL.
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!Chromosomal Pattern
!Chromosomal Pattern
!Molecular Pathogenesis
!Molecular Pathogenesis
!'''Prevalence -'''
!Prevalence -  
'''Common >20%, Recurrent 5-20% or Rare <5% (Disease)'''
Common >20%, Recurrent 5-20% or Rare <5% (Disease)
!'''Diagnostic, Prognostic, and Therapeutic Significance - D, P, T'''
!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T
!'''Established Clinical Significance Per Guidelines - Yes or No (Source)'''
!Established Clinical Significance Per Guidelines - Yes or No (Source)
!'''Clinical Relevance Details/Other Notes'''
!Clinical Relevance Details/Other Notes
|-
|-
|<span class="blue-text">EXAMPLE:</span>
|<span class="blue-text">EXAMPLE:</span>
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|}


<blockquote class='blockedit'>{{Box-round|title=v4:Characteristic Chromosomal Patterns|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>
<blockquote class="blockedit">{{Box-round|title=v4:Characteristic Chromosomal Patterns|The content below was from the previous version of the page. Please incorporate above.}}</blockquote>


Currently there is no specific chromosomal alteration that is characteristic for ETP-ALL.
Currently there is no specific chromosomal alteration that is characteristic for ETP-ALL.
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</blockquote>
</blockquote>
==Gene Mutations (SNV/INDEL)==
==Gene Mutations (SNV/INDEL)==
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: This table is not meant to be an exhaustive list; please include only genes/alterations that are recurrent or common as well either disease defining and/or clinically significant. If a gene has multiple mechanisms depending on the type or site of the alteration, add multiple entries in the table. For clinical significance, denote associations with FDA-approved therapy (not an extensive list of applicable drugs) and NCCN or other national guidelines if applicable; Can also refer to CGC workgroup tables as linked on the homepage if applicable as well as any high impact papers or reviews of gene mutations in this entity. Details on clinical significance such as prognosis and other important information such as concomitant and mutually exclusive mutations can be provided in the notes section. Please include references throughout the table. Do not delete the table.'') </span>
{| class="wikitable sortable"
|-
!Gene!!Genetic Alteration!!Tumor Suppressor Gene, Oncogene, Other!!Prevalence -
Common >20%, Recurrent 5-20% or Rare <5% (Disease)
!Diagnostic, Prognostic, and Therapeutic Significance - D, P, T  
!Established Clinical Significance Per Guidelines - Yes or No (Source)
!Clinical Relevance Details/Other Notes
|-
|<span class="blue-text">EXAMPLE:</span>''EGFR''
<br />
|<span class="blue-text">EXAMPLE:</span> Exon 18-21 activating mutations
|<span class="blue-text">EXAMPLE:</span> Oncogene
|<span class="blue-text">EXAMPLE:</span> Common (lung cancer)
|<span class="blue-text">EXAMPLE:</span> T
|<span class="blue-text">EXAMPLE:</span> Yes (NCCN)
|<span class="blue-text">EXAMPLE:</span> Exons 18, 19, and 21 mutations are targetable for therapy. Exon 20 T790M variants cause resistance to first generation TKI therapy and are targetable by second and third generation TKIs (add references).
|-
|<span class="blue-text">EXAMPLE:</span> ''TP53''; Variable LOF mutations
<br />
|<span class="blue-text">EXAMPLE:</span> Variable LOF mutations
|<span class="blue-text">EXAMPLE:</span> Tumor Supressor Gene
|<span class="blue-text">EXAMPLE:</span> Common (breast cancer)
|<span class="blue-text">EXAMPLE:</span> P
|
|<span class="blue-text">EXAMPLE:</span> >90% are somatic; rare germline alterations associated with Li-Fraumeni syndrome (add reference). Denotes a poor prognosis in breast cancer.
|-
|<span class="blue-text">EXAMPLE:</span> ''BRAF''; Activating mutations
|<span class="blue-text">EXAMPLE:</span> Activating mutations
|<span class="blue-text">EXAMPLE:</span> Oncogene
|<span class="blue-text">EXAMPLE:</span> Common (melanoma)
|<span class="blue-text">EXAMPLE:</span> T
|
|
|-
|
|
|
|
|
|
|
|}Note: A more extensive list of mutations can be found in [https://www.cbioportal.org/ <u>cBioportal</u>], [https://cancer.sanger.ac.uk/cosmic <u>COSMIC</u>], and/or other databases. When applicable, gene-specific pages within the CCGA site directly link to pertinent external content.


Genes encoding transcription factors for development and differentiation (''ETV6, GATA3, HOXA, LMO2, RUNX1, WT1''), kinase signaling (''FLT3, JAK1, JAK3, IL7R, KRAS, NRAS''), and epigenetic modifiers (''DNMT3A, EED, EZH2, PHF6, SUZ12'') are commonly mutated in ETP-ALL <ref name=":5" />. More typical T-ALL mutations, such as ''NOTCH1'' mutations and CDKN1/2 mutations are less frequent in ETP-ALL <ref name=":2" />.
Genes encoding transcription factors for development and differentiation (''ETV6, GATA3, HOXA, LMO2, RUNX1, WT1''), kinase signaling (''FLT3, JAK1, JAK3, IL7R, KRAS, NRAS''), and epigenetic modifiers (''DNMT3A, EED, EZH2, PHF6, SUZ12'') are commonly mutated in ETP-ALL <ref name=":5">{{Cite journal|last=Sin|first=Chun-Fung|last2=Man|first2=Pui-Hei Marcus|date=2021|title=Early T-Cell Precursor Acute Lymphoblastic Leukemia: Diagnosis, Updates in Molecular Pathogenesis, Management, and Novel Therapies|url=https://pubmed.ncbi.nlm.nih.gov/34912707|journal=Frontiers in Oncology|volume=11|pages=750789|doi=10.3389/fonc.2021.750789|issn=2234-943X|pmc=8666570|pmid=34912707}}</ref>. More typical T-ALL mutations, such as ''NOTCH1'' mutations and CDKN1/2 mutations are less frequent in ETP-ALL <ref name=":2">{{Cite journal|last=Jain|first=Nitin|last2=Lamb|first2=Audrey V.|last3=O'Brien|first3=Susan|last4=Ravandi|first4=Farhad|last5=Konopleva|first5=Marina|last6=Jabbour|first6=Elias|last7=Zuo|first7=Zhuang|last8=Jorgensen|first8=Jeffrey|last9=Lin|first9=Pei|date=2016-04-14|title=Early T-cell precursor acute lymphoblastic leukemia/lymphoma (ETP-ALL/LBL) in adolescents and adults: a high-risk subtype|url=https://pubmed.ncbi.nlm.nih.gov/26747249|journal=Blood|volume=127|issue=15|pages=1863–1869|doi=10.1182/blood-2015-08-661702|issn=1528-0020|pmc=4915808|pmid=26747249}}</ref>.


{| class="wikitable sortable"
{| class="wikitable sortable"
|-
|-
!Gene; Genetic Alteration!!'''Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)'''!!'''Prevalence (COSMIC /  TCGA / Other)'''!!'''Concomitant Mutations'''!!'''Mutually Exclusive Mutations'''
!Gene; Genetic Alteration!!Presumed Mechanism (Tumor Suppressor Gene [TSG] / Oncogene / Other)!!Prevalence (COSMIC /  TCGA / Other)!!Concomitant Mutations!!Mutually Exclusive Mutations
!'''Diagnostic Significance (Yes, No or Unknown)'''
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
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==Additional Information==
==Additional Information==


The prognosis of this disease entity was initially considered poor compared to other subtypes of T-ALL based on few small studies <ref name=":10" /><ref name=":3" /><ref>{{Cite journal|last=Ma|first=Meilin|last2=Wang|first2=Xiang|last3=Tang|first3=Jingyan|last4=Xue|first4=Huiliang|last5=Chen|first5=Jing|last6=Pan|first6=Ci|last7=Jiang|first7=Hua|last8=Shen|first8=Shuhong|date=2012-12|title=Early T-cell precursor leukemia: a subtype of high risk childhood acute lymphoblastic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/23065427|journal=Frontiers of Medicine|volume=6|issue=4|pages=416–420|doi=10.1007/s11684-012-0224-4|issn=2095-0225|pmid=23065427}}</ref>. However, more recent studies with larger patient cohorts suggested that the overall outcome with appropriate therapy appeared to not differ significantly from other subtypes <ref>{{Cite journal|last=Patrick|first=Katharine|last2=Wade|first2=Rachel|last3=Goulden|first3=Nick|last4=Mitchell|first4=Chris|last5=Moorman|first5=Anthony V.|last6=Rowntree|first6=Clare|last7=Jenkinson|first7=Sarah|last8=Hough|first8=Rachael|last9=Vora|first9=Ajay|date=2014-08|title=Outcome for children and young people with Early T-cell precursor acute lymphoblastic leukaemia treated on a contemporary protocol, UKALL 2003|url=https://pubmed.ncbi.nlm.nih.gov/24708207|journal=British Journal of Haematology|volume=166|issue=3|pages=421–424|doi=10.1111/bjh.12882|issn=1365-2141|pmid=24708207}}</ref><ref>{{Cite journal|last=Wood|first=Brent L.|last2=Winter|first2=Stuart S.|last3=Dunsmore|first3=Kimberly P.|last4=Devidas|first4=Meenakshi|last5=Chen|first5=Si|last6=Asselin|first6=Barbara|last7=Esiashvili|first7=Natia|last8=Loh|first8=Mignon L.|last9=Winick|first9=Naomi J.|date=2014-12-06|title=T-Lymphoblastic Leukemia (T-ALL) Shows Excellent Outcome, Lack of Significance of the Early Thymic Precursor (ETP) Immunophenotype, and Validation of the Prognostic Value of End-Induction Minimal Residual Disease (MRD) in Children’s Oncology Group (COG) Study AALL0434|url=https://doi.org/10.1182/blood.V124.21.1.1|journal=Blood|volume=124|issue=21|pages=1–1|doi=10.1182/blood.V124.21.1.1|issn=0006-4971}}</ref>.  
The prognosis of this disease entity was initially considered poor compared to other subtypes of T-ALL based on few small studies <ref name=":10">{{Cite journal|last=Coustan-Smith|first=Elaine|last2=Mullighan|first2=Charles G.|last3=Onciu|first3=Mihaela|last4=Behm|first4=Frederick G.|last5=Raimondi|first5=Susana C.|last6=Pei|first6=Deqing|last7=Cheng|first7=Cheng|last8=Su|first8=Xiaoping|last9=Rubnitz|first9=Jeffrey E.|date=2009-02|title=Early T-cell precursor leukaemia: a subtype of very high-risk acute lymphoblastic leukaemia|url=https://pubmed.ncbi.nlm.nih.gov/19147408|journal=The Lancet. Oncology|volume=10|issue=2|pages=147–156|doi=10.1016/S1470-2045(08)70314-0|issn=1474-5488|pmc=2840241|pmid=19147408}}</ref><ref name=":3">{{Cite journal|last=Inukai|first=Takeshi|last2=Kiyokawa|first2=Nobutaka|last3=Campana|first3=Dario|last4=Coustan-Smith|first4=Elaine|last5=Kikuchi|first5=Akira|last6=Kobayashi|first6=Miyuki|last7=Takahashi|first7=Hiroyuki|last8=Koh|first8=Katsuyoshi|last9=Manabe|first9=Atsushi|date=2012-02|title=Clinical significance of early T-cell precursor acute lymphoblastic leukaemia: results of the Tokyo Children's Cancer Study Group Study L99-15|url=https://pubmed.ncbi.nlm.nih.gov/22128890|journal=British Journal of Haematology|volume=156|issue=3|pages=358–365|doi=10.1111/j.1365-2141.2011.08955.x|issn=1365-2141|pmid=22128890}}</ref><ref>{{Cite journal|last=Ma|first=Meilin|last2=Wang|first2=Xiang|last3=Tang|first3=Jingyan|last4=Xue|first4=Huiliang|last5=Chen|first5=Jing|last6=Pan|first6=Ci|last7=Jiang|first7=Hua|last8=Shen|first8=Shuhong|date=2012-12|title=Early T-cell precursor leukemia: a subtype of high risk childhood acute lymphoblastic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/23065427|journal=Frontiers of Medicine|volume=6|issue=4|pages=416–420|doi=10.1007/s11684-012-0224-4|issn=2095-0225|pmid=23065427}}</ref>. However, more recent studies with larger patient cohorts suggested that the overall outcome with appropriate therapy appeared to not differ significantly from other subtypes <ref>{{Cite journal|last=Patrick|first=Katharine|last2=Wade|first2=Rachel|last3=Goulden|first3=Nick|last4=Mitchell|first4=Chris|last5=Moorman|first5=Anthony V.|last6=Rowntree|first6=Clare|last7=Jenkinson|first7=Sarah|last8=Hough|first8=Rachael|last9=Vora|first9=Ajay|date=2014-08|title=Outcome for children and young people with Early T-cell precursor acute lymphoblastic leukaemia treated on a contemporary protocol, UKALL 2003|url=https://pubmed.ncbi.nlm.nih.gov/24708207|journal=British Journal of Haematology|volume=166|issue=3|pages=421–424|doi=10.1111/bjh.12882|issn=1365-2141|pmid=24708207}}</ref><ref>{{Cite journal|last=Wood|first=Brent L.|last2=Winter|first2=Stuart S.|last3=Dunsmore|first3=Kimberly P.|last4=Devidas|first4=Meenakshi|last5=Chen|first5=Si|last6=Asselin|first6=Barbara|last7=Esiashvili|first7=Natia|last8=Loh|first8=Mignon L.|last9=Winick|first9=Naomi J.|date=2014-12-06|title=T-Lymphoblastic Leukemia (T-ALL) Shows Excellent Outcome, Lack of Significance of the Early Thymic Precursor (ETP) Immunophenotype, and Validation of the Prognostic Value of End-Induction Minimal Residual Disease (MRD) in Children’s Oncology Group (COG) Study AALL0434|url=https://doi.org/10.1182/blood.V124.21.1.1|journal=Blood|volume=124|issue=21|pages=1–1|doi=10.1182/blood.V124.21.1.1|issn=0006-4971}}</ref>.  


==Links==
==Links==
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<nowiki>*</nowiki>''Citation of this Page'': “Early T-precursor lymphoblastic leukaemia / lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Early_T-precursor_lymphoblastic_leukaemia_/_lymphoma</nowiki>.
<nowiki>*</nowiki>''Citation of this Page'': “Early T-precursor lymphoblastic leukaemia / lymphoma”. Compendium of Cancer Genome Aberrations (CCGA), Cancer Genomics Consortium (CGC), updated {{REVISIONMONTH}}/{{REVISIONDAY}}/{{REVISIONYEAR}}, <nowiki>https://ccga.io/index.php/HAEM5:Early_T-precursor_lymphoblastic_leukaemia_/_lymphoma</nowiki>.
[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases E]]
[[Category:HAEM5]]
[[Category:DISEASE]]
[[Category:Diseases E]]
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