HAEM5:T-prolymphocytic leukaemia: Difference between revisions
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Atypical lymphocytosis > 5 × 10^9/L | Atypical lymphocytosis > 5 × 10^9/L | ||
Serum lactate dehydrogenase (LDH) (increased) | |||
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==Sites of Involvement== | ==Sites of Involvement== | ||
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!Finding!!Marker | !Finding!!Marker | ||
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|Positive (universal)|| | |Positive (universal)||cyTCL1 (highest specificity), CD2, CD3 (may be weak), CD5, CD7 (strong), TCR-α/β, S100 (30% of cases) | ||
|- | |- | ||
|Positive (subset)||CD4 (in some cases CD4+/CD8+ or CD4-/CD8+), CD52 (therapeutic target), activation markers are variable (CD25, CD38, CD43, CD26, CD27) | |Positive (subset)||CD4 (in some cases CD4+/CD8+ or CD4-/CD8+), CD52 (therapeutic target), activation markers are variable (CD25, CD38, CD43, CD26, CD27) | ||
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|Negative (universal)||TdT, CD1a, CD57, CD16 | |Negative (universal)||TdT, CD1a, CD57, CD16, HTLV1 | ||
|- | |- | ||
|Negative (subset)||CD8 (in some cases CD4+/CD8+ or CD4-/CD8+) | |Negative (subset)||CD8 (in some cases CD4+/CD8+ or CD4-/CD8+) | ||
|} | |} | ||
==Chromosomal Rearrangements (Gene Fusions)== | ==Chromosomal Rearrangements (Gene Fusions)== | ||
Rearrangements involving the TCL1 (T-cell leukemia/lymphoma1) family genes—TCL1A, MTCP1 (mature T-cell proliferation), or TCL1B (also known as TCL1/MTCP1-like 1 [TML1])—are relatively specific to T-PLL. These are present in more than 90% of cases, either as inv(14)(q11q32) or t(14;14)(q11;q32) (involving TCL1A or TCL1B), or t(X;14)(q28;q11) (involving MTCP1). T-PLL-ISG). | |||
{| class="wikitable sortable" | {| class="wikitable sortable" | ||
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|8 | |8 | ||
|Gain | |Gain | ||
|idic(8) | |idic(8)p11 | ||
t(8;8)(p11;q12) | t(8;8)(p11;q12) | ||
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|No | |No | ||
|No | |No | ||
|Recurrent secondary finding (70-80% of cases). Minor diagnostic criteria. <ref>{{Cite journal|last=Staber|first=Philipp B.|last2=Herling|first2=Marco|last3=Bellido|first3=Mar|last4=Jacobsen|first4=Eric D.|last5=Davids|first5=Matthew S.|last6=Kadia|first6=Tapan Mahendra|last7=Shustov|first7=Andrei|last8=Tournilhac|first8=Olivier|last9=Bachy|first9=Emmanuel|date=2019-10-03|title=Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/31292114|journal=Blood|volume=134|issue=14|pages=1132–1143|doi=10.1182/blood.2019000402|issn=1528-0020|pmc=7042666|pmid=31292114}}</ref> | |Recurrent secondary finding (70-80% of cases). Minor diagnostic criteria. <ref>{{Cite journal|last=Staber|first=Philipp B.|last2=Herling|first2=Marco|last3=Bellido|first3=Mar|last4=Jacobsen|first4=Eric D.|last5=Davids|first5=Matthew S.|last6=Kadia|first6=Tapan Mahendra|last7=Shustov|first7=Andrei|last8=Tournilhac|first8=Olivier|last9=Bachy|first9=Emmanuel|date=2019-10-03|title=Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/31292114|journal=Blood|volume=134|issue=14|pages=1132–1143|doi=10.1182/blood.2019000402|issn=1528-0020|pmc=7042666|pmid=31292114}}</ref> | ||
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|5 | |5 | ||
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|Minor diagnostic criteria | |Minor diagnostic criteria | ||
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|6 | |6 | ||
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|Yes | |Yes | ||
|Yes | |Yes | ||
|Frequent, Minor diagnostic criteria. | |Frequent, Minor diagnostic criteria. | ||
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|12 | |12 | ||
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|Yes | |Yes | ||
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| | |Deletions of or missense mutations at the ataxia telangiectasia mutated (''ATM'') locus at 11q23 are found in up to 80% to 90% of T-PLL cases. (T-PLL-ISG) | ||
|- | |- | ||
|''FBXW10'' | |''FBXW10'' | ||