HAEM5:Systemic EBV-positive T-cell lymphoma of childhood: Difference between revisions
| [unchecked revision] | [unchecked revision] |
Karin.Miller (talk | contribs) |
Karin.Miller (talk | contribs) |
||
| Line 159: | Line 159: | ||
== Genetic Diagnostic Testing Methods == | == Genetic Diagnostic Testing Methods == | ||
Acute presentation with fever and systemic symptoms | * WHO 5th edition ''essential'' diagnostic criteria include:<ref name=":3" /><ref name=":6" /> | ||
** 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 | |||
WHO 5th edition ''desirable'' diagnostic criteria include:<ref name=":3" /><ref name=":6" /> | * 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" /> | ||
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" /> | |||
== Familial Forms == | == Familial Forms == | ||