HAEM5:T-prolymphocytic leukaemia: Difference between revisions
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==Definition / Description of Disease== | ==Definition / Description of Disease== | ||
T-prolymphocytic leukemia (T-PLL) is | T-prolymphocytic leukemia (T-PLL) is an aggressive form of T-cell leukemia marked by the proliferation of small to medium-sized prolymphocytes exhibiting a mature post-thymic T-cell phenotype. This condition is characterized by the juxtaposition of TCL1A or MTCP1 genes to a TR locus, typically the TRA/TRD locus. | ||
==Synonyms / Terminology== | ==Synonyms / Terminology== | ||
Put your text here <span style="color:#0070C0">(''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'') </span> | Put your text here <span style="color:#0070C0">(''Instructions: Include currently used terms and major historical ones, adding “(historical)” after the latter.'') </span> | ||
==Epidemiology / Prevalence== | ==Epidemiology / Prevalence== | ||
T-PLL is rare, | T-PLL is a rare disorder, comprising about 2% of all mature lymphoid leukemia cases in adults. It primarily occurs in the elderly, with a median age of 65 years (ranging from 30 to 94 years), and shows a slight male predominance with a male to female ratio of 1.33:1. | ||
==Clinical Features== | ==Clinical Features== | ||
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Marked lymphocytosis > 100 × 10^9/L (75% of cases) | Marked lymphocytosis > 100 × 10^9/L (75% of cases) | ||
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==Sites of Involvement== | ==Sites of Involvement== | ||
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|Positive (universal)||CD2, CD3 (may be weak), CD5, CD7 | |Positive (universal)||CD2, CD3 (may be weak), CD5, CD7 | ||
|- | |- | ||
|Positive (subset)||CD4 (in some cases CD4+/CD8+ or CD4-/CD8+), CD52 | |Positive (subset)||CD4 (in some cases CD4+/CD8+ or CD4-/CD8+), CD52 | ||
|- | |- | ||
|Negative (universal)||TdT, CD1a | |Negative (universal)||TdT, CD1a | ||
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!Notes | !Notes | ||
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|t(14;14)(q11;q32) | |inv(14)(q11q32) | ||
t(14;14)(q11;q32) | |||
|TCL1A/TRD||<span class="blue-text">EXAMPLE:</span> der(22)||<span class="blue-text">EXAMPLE:</span> 20% (COSMIC) | |||
<span class="blue-text">EXAMPLE:</span> 30% (add reference) | <span class="blue-text">EXAMPLE:</span> 30% (add reference) | ||
|Yes | |Yes | ||
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|MTCP1/TRD | |MTCP1/TRD | ||
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|Low | |Low (5%) | ||
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!Notes | !Notes | ||
|- | |- | ||
|8 | |||
|Gain | |||
|idic(8)(p11.2) | |||
t(8;8)(p11.2;q12) | |||
trisomy 8q<br /> | |||
|chr8 | |||
|No | |||
|No | |||
|No | |||
|<span class="blue-text">EXAMPLE:</span> | |<span class="blue-text">EXAMPLE:</span> | ||
Common recurrent secondary finding (70-80% of cases).<ref>{{Cite journal|last=Matutes|first=E.|last2=Brito-Babapulle|first2=V.|last3=Swansbury|first3=J.|last4=Ellis|first4=J.|last5=Morilla|first5=R.|last6=Dearden|first6=C.|last7=Sempere|first7=A.|last8=Catovsky|first8=D.|date=1991-12-15|title=Clinical and laboratory features of 78 cases of T-prolymphocytic leukemia|url=https://pubmed.ncbi.nlm.nih.gov/1742486|journal=Blood|volume=78|issue=12|pages=3269–3274|issn=0006-4971|pmid=1742486}}</ref> | |||
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==Characteristic Chromosomal Patterns== | ==Characteristic Chromosomal Patterns== | ||
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==Familial Forms== | ==Familial Forms== | ||
A subset of cases may develop in the context of ataxia-telangiectasia (AT), which is characterized by germline mutations in the ATM gene. Penetrance of the tumor phenotype is about 10% to 15% by early adulthood.<ref>{{Cite journal|last=Taylor|first=A. M.|last2=Metcalfe|first2=J. A.|last3=Thick|first3=J.|last4=Mak|first4=Y. F.|date=1996-01-15|title=Leukemia and lymphoma in ataxia telangiectasia|url=https://pubmed.ncbi.nlm.nih.gov/8555463|journal=Blood|volume=87|issue=2|pages=423–438|issn=0006-4971|pmid=8555463}}</ref> It represents nearly 3% of all malignancies in patients with ataxia-telangiectasia.<ref>{{Cite journal|last=Li|first=Geling|last2=Waite|first2=Emily|last3=Wolfson|first3=Julie|date=2017-12-26|title=T-cell prolymphocytic leukemia in an adolescent with ataxia-telangiectasia: novel approach with a JAK3 inhibitor (tofacitinib)|url=https://pubmed.ncbi.nlm.nih.gov/29296924|journal=Blood Advances|volume=1|issue=27|pages=2724–2728|doi=10.1182/bloodadvances.2017010470|issn=2473-9529|pmc=5745136|pmid=29296924}}</ref> | |||
==Additional Information== | ==Additional Information== | ||
Put your text here | Put your text here | ||