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Table 1 Patient’s laboratory and clinical findings during his clinical course

From: Successful rescue of a lethal Griscelli syndrome type 2 presenting with neurological involvement and hemophagocytic lymphohistiocytosis: a case report

  Day 0 (at admission) Day 13 (at HLH diagnosis) At the 6th week after HLH treatment Reference Range
Parameters for HLH diagnosis
 High fever + + - -
 Splenomegaly (under ribs, cm) 4 4.5 - -
 Neutrophil count (×109/L) 3.21 1.75 6.08 0.72–4.6
 Platelet count (×109/L) 139 89 116 100–400 × 109
 Hemoglobin (g/L) 82 76 284 110–190
 Fibrinogen (g/L) 2.2 1.24 2.44 2–4
 Triglyceride (mmol/L) 1.52 3.51 1.31 0–2
 Serum ferritin (µg/L) 175.7 269.6 163 28–397
 Soluble CD25(pg/mL) ND 131,840 4117 0–6400
 Hemophagocytosis in BM - ND ND -
 NK cell activity (%) ND 12.81 14.32 >15.44
Other indicators
 Neurological manifestations + +, deterioration - -
 CSF pressure (mmH2O) 230 >330 ND 40–100
 CSF cell count (×106/L) 30 45 ND 0–15 × 106
 CSF Protein (mg/L) 743 1216 ND 20–350
 Head CT scan Low density of brain parenchyma Extensive lesions, cerebral edema, tonsil hernia Greatly improved, cerebral edema (-) tonsil hernia (-) -
 IFN-γ(pg/ml) ND 213.6 9.83 1.60–17.30
 NK cell CD107a (%) ND 1.01 2.33 >10
Infection, malignancy or autoimmune disease - - ND -
  1. +: positive; -: negative
  2. NDnot done