Table 2.
Granzyme B detection in NK and CTL before and after treatment for HLH, prior to BMT.
| HLH etiology | Age of onset HLH | SIl2r(pg/ml) | % GrB+ CD8 | GrB MCF in NK (783 ± 83) | |
|---|---|---|---|---|---|
| P1 | HLHEBV | 8yr | 137,269 | 50 | 2822 |
| 45,231 | 14 | 2862 | |||
| P2 | HLHxlp (BIRC4) | 6 wk | 25,496 | 96 | 2509 |
| 9,410 | 8 | 1681 | |||
| P3 | HLHxlp (BIRC4) | 4 mo | 20,748 | 39 | 3198 |
| 2,078 | 4 | 1231 | |||
| P4 | FHL5 | 12yr | 51,646 | 36 | 2312 |
| 883 | 15 | 833 | |||
| P5 | FHLunk | 7 yr | 5,323 | 74 | 2599 |
| 1,050 | 30 | 970 | |||
| P6 | HLHEBV | 8 yr | 12,280 | 44 | 2173 |
| 900 | 4 | 960 | |||
| P7 | FHL5 | 2 yr | 8,566 | 49 | 1670 |
| 1,002 | 11 | 1137 | |||
| P8 | FHL2 | 5 yr | 15,647 | 69 | 1812 |
| 1,701 | 98 | 986 | |||
| P9 | FHL5 | 4 yr | 29,400 | 81 | 2048 |
| 656 | 3 | 1219 | |||
| P10 | HLHxlp (BIRC4) | 16 mo | 15888 | 64 | 2094 |
| 1876 | 50 | 1061 |
For each patient, serial lab evaluations are displayed by row. Labs that are elevated compared to control values are shaded. The normal control values for sIL2R and GrB in CD8 are age and assay dependent-therefore healthy control values are not listed (See Figure 1 for GrB). The control values for GrB in NK is based on 97 children, aged 0–20 years and represents the 99% confidence interval (parentheses). As >95% of NK cells in patients are GrB (+), only the variable value of MCF is shown.