Table 2.

Hemophagocytic lymphohistiocytosis clinical features and laboratory results in MAS and non-MAS patients by cohort.

Cohort 1, n = 34Cohort 2, n = 41
MAS, n = 10Non-MAS, n = 24MAS, n = 10Non-MAS, n = 31
Clinical features, n (%)
    Lymphadenopathy7 (70)15 (63)5 (50)15 (47)
    Hepatomegaly2 (20)4 (17)2 (20)5 (16)
    Splenomegaly1 (10)7 (29)5 (50)7 (22)
    CNS4 (40)6 (25)3 (30)12 (38)
    Bleeding1 (10)7 (29)3 (30)7 (22)
    Hemophagocytosis on BMa3/6 (50)3/14 (21)2/9 (22)1/14 (7)
Laboratory findings, mean ± SD
    Hemoglobin, g/L97.3 ± 18.687.8 ± 14.396.4 ± 18.594.2 ± 16.7
    WBC, × 109/L2.6 ± 1.75.1 ± 3.02.2 ± 1.34.4 ± 2.6
    Neutrophils, × 109/L1.31 ± 0.962.89 ± 2.371.10 ± 0.642.65 ± 1.68
    Lymphocytes, × 109/L0.62 ± 0.341.29 ± 0.670.59 ± 0.401.22 ± 0.69
    Platelet count, × 109/L158 ± 127192 ± 176115 ± 54210 ± 144
    AST, U/L163 ± 17167 ± 56b190 ± 20478 ± 119
    ALT, U/L78 ± 6937 ± 24b77 ± 5769 ± 155
    LDH, U/L2094 ± 13481055 ± 443 (n = 18)2046 ± 1019b1167 ± 1157c
    Albumin, g/L27 ± 931 ± 8c28 ± 7b28 ± 7b
    Ferritin, ug/L7579 ± 16,647b757 ± 1282 (n = 16)2796 ± 2164b808 ± 1591 (n = 25)
    Fibrinogen, g/L3.3 ± 1.44.3 ± 1.6 (n = 11)2.7 ± 0.74.1 ± 1.5 (n = 22)
    D-dimer, μg/mL4.2 ± 10.11.26 ± 0.964.8 ± 2.83.2 ± 4.1 (n = 23)
    Triglycerides, mmol/L2.2 ± 1.52.4 ± 1.34 (n = 20)3.2 ± 1.23b2.3 ± 1.9 (n = 27)
    INR1.1 ± 0.21.0 ± 0.4b1.1 ± 0.11.0 ± 0.1b
    aPTT, sec37 ± 831 ± 4b35 ± 732 ± 7b
    C3, g/L0.43 ± 0.330.59 ± 0.490.49 ± 0.480.62 ± 0.33
    C4, g/L0.086 ± 0.0840.095 ± 0.0850.082 ± 0.1160.100 ± 0.085
    Urea, mmol/L7.0 ± 5.65.6 ± 3.173.71 ± 1.735.28 ± 3.89b
    Creatinine, μmol/L88 ± 5369 ± 3367 ± 2266 ± 41
    Calcium, mmol/L1.90 ± 0.372.04 ± 0.161.96 ± 0.212.05 ± 0.29
    Sodium, mmol/L139 ± 5136 ± 5136 ± 5b139 ± 3c
    ESR, mm/h81 ± 43102 ± 22c70 ± 52100 ± 37
    CRP, mg/L21.8 ± 39.7 (n = 8)27.0 ± 48.4c30.6 ± 34.325.4 ± 40.4b
    IgG, g/L19.5 ± 8.1 (n = 7)20.2 ± 5.9 (n = 21)18.3 ± 4.222.3 ± 10.3b
  • a BM available in a subset of patients. Number reported in each group.

  • b Laboratory data missing on 1 subject.

  • c Laboratory data missing on 2 subjects. ALT: alanine aminotransferase; aPTT: activated partial thromboplastin time; AST: aspartate aminotransferase; BM: bone barrow; CNS: central nervous system; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; LDH: lactate dehydrogenase; INR: international normalized ratio; MAS: macrophage activation syndrome; WBC: white blood cell.