HAEM5:T-prolymphocytic leukaemia: Difference between revisions

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!Clinical Relevance Details/Other Notes
!Clinical Relevance Details/Other Notes
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|inv(14)<br />||TCRα/δ and TCL1A||Pericentric inversion within chromosome 14, leading to '''j'''uxtaposition of the ''TCRα/δ'' enhancer to the ''TCL1A'' locus and aberrant overexpression of ''TCL1A''||inv(14)(q11.2q32.1)
|inv(14)<br />||TCRα/δ and TCL1A||Pericentric inversion within chromosome 14, leading to '''j'''uxtaposition of the ''TCRα/δ'' enhancer to the ''TCL1A'' locus and aberrant overexpression of ''TCL1A''||inv(14)(q11.2q32.1)*
|Common ~60%
|Common ~60%
|<span class="blue-text">EXAMPLE:</span> D, P, T
|D
|<span class="blue-text">EXAMPLE:</span> Yes (WHO, NCCN)
|No
|These genetic abnormalities serve as diagnostic markers and generally indicate an aggressive disease.  Major diagnostic criteria.<ref name=":6" />
|These genetic abnormalities serve as diagnostic markers and generally indicate an aggressive disease.  Major diagnostic criteria.<ref name=":6" />
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|TCRα/δ and TCL1A
|TCRα/δ and TCL1A
|''Reciprocal translocation between the two'' homologous chromosome 14s, leading to juxtaposition of the ''TCRα/δ'' enhancer to the ''TCL1A'' locus  and aberrant overexpression of ''TCL1A''
|''Reciprocal translocation between the two'' homologous chromosome 14s, leading to juxtaposition of the ''TCRα/δ'' enhancer to the ''TCL1A'' locus  and aberrant overexpression of ''TCL1A''
|t(14;14)(q11.2;q32.1)
|t(14;14)(q11.2;q32.1)*
|Recurrent ~20%
|Recurrent ~20-25%
|<span class="blue-text">EXAMPLE:</span> D
|D
|
|No
|<span class="blue-text">EXAMPLE:</span>
|*In most diagnostic/genetic reports (e.g., FISH or karyotype), the inversion and translocation may grouped together. Their distinction is mainly cytogenetic, not biological.
 
''DUX4'' has many homologous genes; an alternate translocation in a minority of cases is t(10;19), but this is usually indistinguishable from t(4;19) by short-read sequencing (add references).
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|t(X;14)
|t(X;14)
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|t(X;14)(q28;q11.2)
|t(X;14)(q28;q11.2)
|Rare ~5%
|Rare ~5%
|
|D
|
|No
|Major diagnostic criteria.<ref name=":6" />
|Major diagnostic criteria.<ref name=":6" /> Rarely, t(X;7)(q28;q34) is observed, where the TCRβ enhancer (7q34) substitutes for TCRα/δ, leading to the same functional outcome
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{| class="wikitable sortable"
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!Chromosomal Rearrangement!!Genes in Fusion (5’ or 3’ Segments)!!Pathogenic Derivative!!Prevalence
!Diagnostic Significance (Yes, No or Unknown)
!Prognostic Significance (Yes, No or Unknown)
!Therapeutic Significance (Yes, No or Unknown)
!Notes
|-
|inv(14)(q11.2q32.1)
t(14;14)(q11.2;q32.1)
|''TCL1A/B ,TRD''|| ||inv(14) ~60%
t(14;14) ~25%
|Yes
|No
|No
|
|-
|t(X;14)(q28;q11.2)
|''MTCP1, TRD''
|
|Low (5%)
|Yes
|No
|No
|Major diagnostic criteria.<ref name=":6" />
|}
==Individual Region Genomic Gain/Loss/LOH==
==Individual Region Genomic Gain/Loss/LOH==
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene rearrangements. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Can refer to CGC workgroup tables as linked on the homepage if applicable. Please include references throughout the table. Do not delete the table.'') </span>
Put your text here and fill in the table <span style="color:#0070C0">(''Instructions: Includes aberrations not involving gene rearrangements. Details on clinical significance such as prognosis and other important information can be provided in the notes section. Can refer to CGC workgroup tables as linked on the homepage if applicable. Please include references throughout the table. Do not delete the table.'') </span>