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" /> | |||
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. 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 | ||