HAEM5:Systemic chronic active EBV disease: Difference between revisions

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==Genetic Diagnostic Testing Methods==
==Genetic Diagnostic Testing Methods==


* The WHO 5<sup>th</sup> edition essential diagnostic criteria include:<ref name=":0" />
* The WHO 5<sup>th</sup> edition essential diagnostic criteria include:<ref name=":0" /><ref name=":5">The WHO Classification of Tumours Editorial Board, ed. ''Haematolymphoid Tumours: Who Classification of Tumours''. 5th ed. International Agency for Research on Cancer; 2024.</ref>
** Persistent (greater than 3 months) infectious mononucleosis-like symptoms
** Persistent (greater than 3 months) infectious mononucleosis-like symptoms
** Increased EBV DNA in the peripheral blood or EBER-positive cells in affected organs with evidence of EBV infection in T or NK-cells
** Increased EBV DNA in the peripheral blood or EBER-positive cells in affected organs with evidence of EBV infection in T or NK-cells
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** Exclusion of known immunodeficiency, malignancy, or autoimmune disorders
** Exclusion of known immunodeficiency, malignancy, or autoimmune disorders


* '''Both the WHO 5<sup>th</sup> edition and ICC include detection of increased EBV DNA in the peripheral blood and/or EBV RNA (EBER) or viral protein in T or NK-cells of affected tissues.<ref name=":0" /><ref name=":1" />'''
* '''Both the WHO 5<sup>th</sup> edition and ICC include detection of increased EBV DNA in the peripheral blood and/or EBV RNA (EBER) or viral protein in T or NK-cells of affected tissues.'''<ref name=":5" />'''<ref name=":0" /><ref name=":1" />'''
** Whole blood or peripheral blood mononuclear cells are preferred for EBV DNA PCR testing, as serum or plasma are less sensitive for CAEBV disease<ref>{{Cite journal|last=Kimura|first=Hiroshi|last2=Cohen|first2=Jeffrey I.|date=2017|title=Chronic Active Epstein-Barr Virus Disease|url=https://pubmed.ncbi.nlm.nih.gov/29375552|journal=Frontiers in Immunology|volume=8|pages=1867|doi=10.3389/fimmu.2017.01867|issn=1664-3224|pmc=5770746|pmid=29375552}}</ref>
** Whole blood or peripheral blood mononuclear cells are preferred for EBV DNA PCR testing, as serum or plasma are less sensitive for CAEBV disease<ref>{{Cite journal|last=Kimura|first=Hiroshi|last2=Cohen|first2=Jeffrey I.|date=2017|title=Chronic Active Epstein-Barr Virus Disease|url=https://pubmed.ncbi.nlm.nih.gov/29375552|journal=Frontiers in Immunology|volume=8|pages=1867|doi=10.3389/fimmu.2017.01867|issn=1664-3224|pmc=5770746|pmid=29375552}}</ref>
** In tissues, using a double stain for B, T, or NK-cell markers and EBV is recommended.
** In tissues, using a double stain for B, T, or NK-cell markers and EBV is recommended.