Table 2.

Patients with a diagnosis of EBV-associated HLH

PatientAge/sexNK LU% NK% perforin
NKCD8CD56
P8 1 y/M  4.3 <1↓ 88 81↑ 32 
P5 2 y/M NA 1↓ 96 26↑ 24 
P4 4 y/M 0.02↓ <1↓ 82 9  28 
P3 11 y/M 0.00↓ 3↓ 85 66↑ 76↑ 
Normal values 1-15 y >3.2 4-22 81-91 2-11 12-34 
PatientAge/sexNK LU% NK% perforin
NKCD8CD56
P8 1 y/M  4.3 <1↓ 88 81↑ 32 
P5 2 y/M NA 1↓ 96 26↑ 24 
P4 4 y/M 0.02↓ <1↓ 82 9  28 
P3 11 y/M 0.00↓ 3↓ 85 66↑ 76↑ 
Normal values 1-15 y >3.2 4-22 81-91 2-11 12-34 

Patients with EBV-associated HLH were generally older at diagnosis and were more likely to have very low numbers of NK cells and increased perforin-expressing CD8+ T cells. Perforin-expressing CD8+ T cells of patient P4 did not show an increased value, but the specimen of this patient was drawn a couple of weeks after initiation of therapy.

NA indicates not applicable.