Compendium of Cancer Genome Aberrations

HAEM5:Systemic EBV-positive T-cell lymphoma of childhood: Difference between revisions

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|The WHO 5th edition notes that diagnosis of SEBVTCL of childhood "may be supported by clonal TR gene rearrangements," and clonal TR gene rearrangements are included as desirable diagnostic criteria.<ref>The WHO Classification of Tumours Editorial Board, ed. ''Haematolymphoid Tumours: Who Classification of Tumours''. 5th ed. International Agency for Research on Cancer; 2024.</ref>  
|The WHO 5th edition notes that diagnosis of SEBVTCL of childhood "may be supported by clonal TR gene rearrangements," and clonal TR gene rearrangements are included as desirable diagnostic criteria.<ref name=":3">The WHO Classification of Tumours Editorial Board, ed. ''Haematolymphoid Tumours: Who Classification of Tumours''. 5th ed. International Agency for Research on Cancer; 2024.</ref><ref name=":6">{{Cite journal|title=BlueBooksOnline|url=https://tumourclassification.iarc.who.int/chapters/63}}</ref>
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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==


*Morphology and immunophenotyping (IHC or flow cytometry)
*WHO 5th edition ''essential'' diagnostic criteria include:<ref name=":3" /><ref name=":6" />
*Clonal proliferation of T cells (polyclonal cases have been reported<ref>{{Cite journal|last=Chen|first=Guoshu|last2=Chen|first2=Li|last3=Qin|first3=Xiaohua|last4=Huang|first4=Zhuoya|last5=Xie|first5=Xiaoling|last6=Li|first6=Guowei|last7=Xu|first7=Bing|date=2014|title=Systemic Epstein-Barr virus positive T-cell lymphoproliferative disease of childhood with hemophagocytic syndrome|url=https://pubmed.ncbi.nlm.nih.gov/25400806|journal=International Journal of Clinical and Experimental Pathology|volume=7|issue=10|pages=7110–7113|issn=1936-2625|pmc=4230111|pmid=25400806}}</ref>)
**Acute presentation with fever and systemic symptoms
**Multiorgan infiltration by atypical T-cells
**EBV-positivity exclusion of known immunodeficiency
*WHO 5th edition ''desirable'' diagnostic criteria include:<ref name=":3" /><ref name=":6" />
**'''Clonal TCR-gene rearrangement'''
**Hemophagocytic lymphohistiocytosis (HLH)
**Hepatosplenomegaly
*TCR-gene rearrangements can be detected via PCR or NGS methods


==Familial Forms==
==Familial Forms==
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