HLH diagnostic criteria | Patient laboratory values | |||
---|---|---|---|---|
Timing relative to anakinra trial | Admission to prior to anakinra | During anakinra (day from start of anakinra) | One week after anakinra | |
Cytopenia in ≥ 2 cell lines | ||||
Haemoglobin < 9g/dL (90g/L) | WNL | 8.8 (d2); Nadir 5.2 (d16) | WNL | |
Platelet < 100 × 109/L | 60 | 26 (d 16) | 13-21 | |
Absolute neutrophil count < 1000 / μL | 924 | 700 (d17) | 48-153 | |
Hypertriglyceridemia and/or Hypofibrinogenemia | ||||
Fasting triglyceride ≥ 265 mg/dL) | - | 802 (d16) | 423, 577, 726 | |
Fibrinogen < 150 mg/dL | 127 | WNL | - | |
Ferritin ≥ 500 ng/mL (500 µg/L) | 905 | 629 (d15) | 4814, 7597 | |
sCD25 ≥ 2400 μg/mL | - | 42,140 (d17) | - | |
Decreased or absent NK cell activity | ||||
LU20 NK a | 108 | 37.4 (d4) | - | |
CD107a b | - | 7 % (d17) | - | |
Hemophagocytosis in bone marrow | “some” | “numerous” |