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

[[HAEM5:Table_of_Contents|Haematolymphoid Tumours (5th ed.)]]

<blockquote class='blockedit'>{{Box-round|title=HAEM5 Conversion Notes|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]].

 

==Cancer Category / Type==

 

[https://ccga.io/index.php/HAEM4:T-Lymphoblastic_Leukemia/Lymphoma T-Lymphoblastic Leukemia (T-ALL)]

==Cancer Sub-Classification / Subtype==

|'''Signs and Symptoms'''

|EXAMPLE Asymptomatic (incidental finding on complete blood counts)

 

 

 

|'''Laboratory Findings'''

|EXAMPLE Cytopenias

 

!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" />

Put your text here and fill in the table

!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

|EXAMPLE t(9;22)(q34;q11.2)||EXAMPLE 3'ABL1 / 5'BCR||EXAMPLE der(22)||EXAMPLE 20% (COSMIC)

|Yes

|No

|Yes

|EXAMPLE

 

|}

<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.}}

==Individual Region Genomic Gain / Loss / LOH==

!Chr #!!Gain / Loss / Amp / LOH!!Minimal Region Genomic Coordinates [Genome Build]!!Minimal Region Cytoband

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

!Therapeutic Significance (Yes, No or Unknown)

!Notes

|EXAMPLE

 

|EXAMPLE Loss

|EXAMPLE

 

chr7:1- 159,335,973 [hg38]

 

|Yes

|Yes

|No

|EXAMPLE

 

Presence of monosomy 7 (or 7q deletion) is sufficient for a diagnosis of AML with MDS-related changes when there is ≥20% blasts and no prior therapy (add reference).  Monosomy 7/7q deletion is associated with a poor prognosis in AML (add reference).

|EXAMPLE

 

|EXAMPLE Gain

|EXAMPLE

 

chr8:1-145,138,636 [hg38]

 

|No

|No

|No

|EXAMPLE

 

Common recurrent secondary finding for t(8;21) (add reference).

<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.}}

==Characteristic Chromosomal Patterns==

!Diagnostic Significance (Yes, No or Unknown)

!Prognostic Significance (Yes, No or Unknown)

!Therapeutic Significance (Yes, No or Unknown)

!Notes

|EXAMPLE

 

|Yes

|EXAMPLE:

 

See chromosomal rearrangements table as this pattern is due to an unbalanced derivative translocation associated with oligodendroglioma (add reference).

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

==Gene Mutations (SNV / INDEL)==

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" />.

!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)'''

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>.  

[[Category:HAEM5]][[Category:DISEASE]][[Category:Diseases E]]