On Two Cases with Autosomal Dominant Hyper IgE Syndrome: Importance of Immunological Parameters for Clinical Course and Follow-Up
Table 3
Immunological phenotype of Patients 1 and 2.
Immune phenotype/marker (units)
Patient 1
Patient 2
Reference range
WBC (cells × 109/L)
7.3
7.0
4.5 ÷ 13
ANC (cells × 109/L)
1.97
2.0
1.8 ÷ 8.0
ALC (cells × 109/µL)
3.79
3.8
1.5 ÷ 6.5
Eos (%)
12
10.2
0.0 ÷ 6.0
CD3+ (%Ly)
63
77
66 ÷ 76
CD3+DR+ (%Ly)
9
5
9.5 ÷ 17
CD3+CD4+ (%Ly)
36
45
33 ÷ 41
CD45RA+62L+ from CD4+ (%Ly)
24.8
73.6
46 ÷ 99
CD45RA-62L+ from CD4+ (%Ly)
6.4
19.8
0.35 ÷ 100
CD45RA-62L− from CD4+ (%Ly)
24.8
4.8
0.27 ÷ 18
CD45RA+62L− from CD4+ (%Ly)
44.0
1.8
<1.8
CD3+CD8+ (%Ly)
19
21
27 ÷ 35
CD45RA+62L+ from CD8+ (%Ly)
45.5
68.1
16 ÷ 100
CD45RA-62L+ from CD8+ (%Ly)
5.6
5.9
1 ÷ 6
CD45RA-62L- from CD8+ (%Ly)
23.6
12.4
5 ÷ 100
CD45RA+62L- from CD8+ (%Ly)
25.3
13.6
15 ÷ 41
CD19+ (%Ly)
18
20
12 ÷ 22
CD3-CD16 + 56 (%Ly)
14
6
9 ± 16
CD3+CD16 + 56+ (%Ly)
10
3
4 ÷ 26
CD25+CD127low (%Ly)
8.4
6.2
5 ÷ 10
CD4+CD161+CD196+ (%Ly)
2.34; 2.0
1.2
12.5 ÷ 14.9
IgG (g/l)
16.079
10.64
5.40–16.10
IgG1(g/l)
10.902
8.482
4.23–10.6
IgG2 (g/l)
2.5
2.738
0.72–4.3
IgG3 (g/l)
0.681
0.934
0.127–1.731
IgG4 (g/l)
0.428
0.24
0.016–1.151
IgA (g/l)
1,742
0.75
0.50–2.80
IgM (g/l)
1.400
2.41
0.5–1.90
IgE (U/ml)
2180; 1995; 1104; 1414; 2000
431; >2500; 9740; 22400
<87
Aspergillus fumigates-specific IgE
Negative
Positive
Negative
C3 (g/l)
1,557
1.303
0.75–1.65
C4 (g/l)
0.510
0.118
0.20–0.65
ANA (U/ml)
1 : 160
1 : 160
1 : 160
ASO (U/ml)
1066; 522 U/ml
12
<200
PCP IgG (mg/L)
85.6
15.4
>30
PCP IgG2 (mg/L)
32.0
2.88
>11
PCP IgA (mg/L)
1.03
74.6
NA
PCP IgM (mg/L)
3.53
209.4
NA
Hib IgG (mg/L)
16.5
6.43
>0.15
DT IgG (mg/L)
0.04
0.025
>0.1
TT IgG (mg/L)
0.11
0.35
>0.1
Low values; high values; measurement of IgE is in flux with approximately one-year follow-up intervals. WBC: white blood cell count; ANC: absolute neutrophil count; ALC: absolute lymphocyte count; Eos: eosinophils; PCP: pneumococcal capsular polysaccharide; Hib: Haemophilus influenzae type B; DT: diphtheria toxoid; Td: tetanus toxoid; NA: not applicable. The estimation of PCP IgA and IgM values was based on the comparison with the titer of the same antibodies in children tested in our laboratory (data not published).
