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Table 1 Laboratory values meeting HLH diagnostic criteria found prior to, during and after trial of anakinra

From: Presentation of hemophagocytic lymphohistiocytosis due to a novel MUNC 13–4 mutation masked by partial therapeutic immunosuppression

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”
  1. -: laboratory value not measured; d: day from start of anakinra; NK: natural killer; WNL: within normal limits.
  2. a LU20 NK: Patient NK cells were cultured with radioisotope-labeled tumor cells. Functional NK cell activity measured by quantifying released isotope after correction for patient peripheral blood mononuclear cell % NK.
  3. b CD107a: Mononuclear cells from peripheral blood were stimulated with K562 target cells to induce degranulation. Six hours post incubation, CD107a expression was detected by flow cytometry.