Table 2.
Laboratory evaluation of chronic granulomatous disease (CGD) patients with hemophagocytic lymphohistiocytosis (HLH).
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | |||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Day 8 | Day 9 | Day 10 | Day 6 | Day 7 | Day 8 | At HLH diagnosis | Day 1 | Day 3 | Day 30 | Day 31 | Day 34 | |
| Associated infections | Burkholderia cepacia pneumonia and sepsis | Penicillium pneumonia | Disseminated Candida lusitaniae † | Burkholderia multivorans sepsis | ||||||||
| WBC (6–14 k/μl) | 10.7 | 2.49 | 1.42 | 2.6 | 4.5 | 5.5 | 1.6 | 4.8 | 4.6 | 7.0 | 7.5 | 11.5 |
| Hemoglobin (10.5–14 g/dl) | 8.7 | 6.9 | 7.7* | 7.8 | 10 | 8.7 | 5.9 | 8.7 | 8.8 | 10.7 | 12.2 | 10.9 |
| Platelet (150–450 k/μl) | 195 | 96 | 18 | 37 | 72 | 53 | 90 | 170 | 162 | 64 | 84 | 66 |
| ANC (1,100–6,600) | 6,350 | 1,050 | 90 | 3,570 | 3,590 | |||||||
| CRP (<1 mg/dl) | 25.4 | 11.7 | 13.7 | 15 | 21.2 | |||||||
| ALT (13–50 IU/L) | 141 | 216 | 307 | 50 | 74 | 123 | 53 | 54 | 65 | 703 | ||
| AST (22–63 IU/L) | 266 | 656 | 1670 | 245 | 495 | 646 | 86 | 64 | 98 | 3168 | ||
| INR (0.9–1.1) | 1.32 | 2.77 | 3.73 | 1.5 | 1.4 | 1.5 | 1.4 | 2.4 | 2.6 | |||
| Fibrinogen (159–480 mg/dl) | 273 | 135 | 108+ | 116 | 117 | 70 | 80 | 529 | 312 | 322 | 272 | |
| Lactate (0.6–1.1mmol/L) | 6.4 | >20 | 5.4 | 2.6 | ||||||||
| Triglyceride (≤149 mg/dl) | 180 | 196 | 211 | 791 | ||||||||
| Ferritin (22–322 μg/L) | 11,531 | >16,500 | >16,500 | 11,783 | 2,554 | 2,393 | 34,075 | 65,027 | >100,000 | |||
| Soluble IL-2R (<2,400 U/ml) | 23,544 | 18,857 | 17,035 | 16,876 | ||||||||
| CXCL9 (≤121 pg/ml) | 27,292 | 123.015¶ | ||||||||||
*s/p pRBCs, +s/p FFP, cryoprecipitate, ¶collected on day 23, †after treatment for HLH.