TABLE I.
Clinical characteristics
ID | Sex | Viruses eliciting severe disease | HLH or HLH-like disease | Organs affected | Age at last FU | Outcome | |||
---|---|---|---|---|---|---|---|---|---|
Liver | CNS | Kidney | Lung | ||||||
P1.1 | F | — | − | + | − | − | + | 2 y 8 mo | Dead |
P1.2 | F | Influenza A | − | + | + | + | + | 15 y | Dead |
P1.3 | M | Influenza A | + | + | + | − | + | 1 y 2 mo | Dead |
P2.1 | F | — | − | + | − | + | + | 14 y | Alive |
P2.2 | M | RSV Influenza A | + | + | (+) | + | + | 3 mo | Dead |
P3.1 | F | NA | NA | NA | + | NA | NA | 5 mo | Dead |
P3.2 | M | Norovirus HHV6 | + | + | + | + | + | 1 y | Dead |
P4.1 | F | ADV | + | + | + | + | + | 8 mo | Dead |
P4.2 | M | ADV Parainfluenza | + | + | + | + | − | 8 y | Alive |
P5.1 | M | HHV6 (+CMV) | + | + | + | + | + | 3 mo | Dead |
P5.2 | F | HHV6 (+Sapovirus, rhinovirus) | + | + | − | + | + | 1 y 4 mo | Dead |
P6.1 | M | Sepsis, germ not identified | − | + | − | + | + | 9 y | Dead |
P7.1 | F | Vaccine strain measles (+EBV) Influenza B | + | + | + | + | + | 3 y | Alive |
P8.1 | F | CMV | + | + | + | + | + | 8y | Dead |
P8.2 | M | Vaccine strain VZV Influenza A | + | + | + | − | + | 7 y | Alive |
Summary | F/M, 8:7 | Negative ssRNA viruses: 10 Positive ssRNA viruses: 2 dsRNA viruses: 1 dsDNA viruses: 7 |
n = 10 | n = 14 | n = 11 | n = 11 | n = 13 | Alive/dead 4:11 |
ADV, Adenovirus; CMV, cytomegalovirus; CNS, central nervous system; dsDNA, double-stranded DNA; F, female; FU, follow-up; HHV6, human herpes virus type 6; HLH, hemophagocytic lymphohistiocytosis; ID, identifier; M, male; NA, not available; RSV, respiratory syncytial virus; SAP, SLAM-associated protein; VZV, varicella zoster virus.