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

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== Genetic Diagnostic Testing Methods ==
== Genetic Diagnostic Testing Methods ==
WHO 5th edition ''essential'' diagnostic criteria include:<ref name=":3" /><ref name=":6" />


* WHO 5th edition ''essential'' diagnostic criteria include:<ref name=":3" /><ref name=":6" />
Acute presentation with fever and systemic symptoms
** 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" />
Multiorgan infiltration by atypical T-cells
** Clonal TCR-gene rearrangement
** Hemophagocytic lymphohistiocytosis (HLH)
** Hepatosplenomegaly


* TCR-gene rearrangements can be detected via PCR or NGS methods. Of note, T-cell clonality can also be detected in EBV-associated HLH and other EBV-associated disorders.<ref name=":10" />
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. Of note, T-cell clonality can also be detected in EBV-associated HLH and other EBV-associated disorders.<ref name=":10" /><center>
== Familial Forms ==
== Familial Forms ==
Racial predisposition suggests a genetic background; however, no specific genetic abnormalities have been detected
Racial predisposition suggests a genetic background; however, no specific genetic abnormalities have been detected