|
(a) Clinical and biological characteristics
|
|
Age (mean, SD)
|
60.0 (10.3)
|
60.4 (10.5)
|
0.91
|
|
Sex (male, %)
|
13 (72%)
|
13(72%)
|
1
|
|
Mutation (n, %)1
|
|
- ENG
|
11 (61%)
|
7 (39%)
|
0.49
|
|
- ACVRL1
|
7 (39%)
|
9 (50%)
| |
|
Epistaxis frequency (n, %)
|
|
- Daily
|
6 (34%)
|
7 (39%)
|
0.56
|
|
- Weekly
|
8 (44%)
|
5 (28%)
| |
|
- Monthly or more rarely
|
4 (22%)
|
6 (34%)
| |
|
Visible telangiectases (median, min–max)
|
58 (0–242)
|
90 (0–200)
|
0.39
|
|
Arteriovenous malformations (n, %2)
|
|
- Lung3
|
9 (50%)
|
3 (18%)
|
0.044
|
|
- Digestive tract
|
7 (63%)
|
12 (80%)
|
0.35
|
|
- Liver
|
8 (44%)
|
7 (44%)
|
0.97
|
|
- Brain
|
5 (29%)
|
1 (14%)
|
0.78
|
|
Treatment (n, %)
|
|
- IV iron or blood transfusion (at least 5)
|
8 (44%)
|
12 (67%)
|
0.14
|
|
- Tranexamic acid
|
2 (11%)
|
4 (22%)
|
0.37
|
|
- Bevacizumab
|
1 (6%)
|
3 (17%)
|
0.29
|
|
Basic biological parameters (median, min–max):
|
|
Hemoglobin (g/l)
|
135 (80–188)
|
126 (78–157)
|
0.16
|
|
Platelets (G/l)
|
245 (178–388)
|
262 (236–488)
|
0.15
|
|
Leucocytes (G/l)
|
5.44 (3.00–8.92)
|
5.23 (3.02–10.23)
|
0.95
|
|
Neutrophils (G/l)
|
4.06 (1.73–7.65)
|
4.02 (1.94–8.19)
|
0.67
|
|
Lymphocytes (G/l)
|
0.92 (0.3–2.4)
|
1.02 (0.4–2.34)
|
0.89
|
|
C reactive protein (mg/l)
|
2.63 (0.2–8.4)
|
1.85 (0.2–7.3)
|
0.63
|
|
Ferritin (ng/ml)
|
76 (9–158)
|
57 (7–221)
|
0.43
|
|
Creatinine clearance (MDRD, ml/min)
|
85 (36–107)
|
86.5 (42–140)
|
0.52
|
|
Immunoglobulin G (g/l)
|
8,73 (2.7–16.8)
|
9.16 (3.16–16.9)
|
0.87
|
|
Immunoglobulin A (g/l)
|
2.10 (1.04–4.3)
|
2.23 (1.07–4.06)
|
0.64
|
|
Immunoglobulin M (g/l)
|
0.69 (0.36–1.79)
|
0.72 (0.19–1.9)
|
0.97
|
|
(b) Lymphocyte parameters (median, min–max)
|
|
T lymphocytes (cells/mm3)
|
841 (219–2116)
|
829 (214–1831)
|
0.44
|
|
T-helper lymphocytes (cells/mm3)
|
517 (96–1499)
|
550 (123–1061)
|
0.83
|
|
T-cytotoxic lymphocytes (cells/mm3)
|
339 (90–848)
|
187 (51–1110)
|
0.12
|
|
NK lymphocytes (cells/mm3)
|
211 (12–989)
|
170 (55–946)
|
0.64
|
|
Naive T-helper lymphocytes (cells/mm3)
|
273 (8–978)
|
201 (14–630)
|
0.55
|
|
Memory T-helper lymphocytes (cells/mm3)
|
236 (79–519)
|
280 (109–798)
|
0.60
|
|
Activated T-helper lymphocytes (cells/mm3)
|
59 (19–151)
|
58 (30–215)
|
0.78
|
|
CXCR4 MFI on T lymphocytes
|
3,87 (2.17–18.36)
|
3.74 (2.20–14.30)
|
0.80
|
|
CXCR4 MFI on T-helper lymphocytes
|
4.80 (2.91–14.00)
|
4.17 (2.40–8.20)
|
0.48
|
|
CXCR4 MFI on Naive T-helper lymphocytes
|
9.65 (4.48–35.00)
|
7.01 (3.34–14.20)
|
0.03
|
|
CXCR4 MFI on memory T-helper lymphocytes
|
3.06 (2.28–9.79)
|
3.33 (1.91–6.00)
|
0.64
|
|
CXCR4 MFI on activated T-helper lymphocytes
|
2.40 (1.71–8.15)
|
2.49 (1.63–4.00)
|
0.97
|
|
CD26 MFI on T lymphocytes
|
2.71 (1.41–5.13)
|
2.90 (2.21–6.54)
|
0.13
|
|
CD26 MFI on T-helper lymphocytes
|
3.01 (1.72–5.75)
|
3.26 (2.17–6.92)
|
0.19
|
|
CD26 MFI on naive T-helper lymphocytes
|
4.59 (1.65–9.33)
|
5.73 (4.17–14.1)
|
0.03
|
|
CD26 MFI on memory T-helper lymphocytes
|
2.27 (1.58–3.48)
|
2.58 (2.00–5.08)
|
0.09
|
|
CD26 MFI on activated T-helper lymphocytes
|
1.63 (1.32–2.95)
|
1.57 (1.13–2.77)
|
0.29
|
|
CXCR4/CD26 ratio on T lymphocytes
|
1.63 (0.52–5.45)
|
1.32 (0.45–4.38)
|
0.04
|
|
CXCR4/CD26 ratio on T-helper lymphocytes
|
1.58 (0.86–3.41)
|
1.37 (0.62–2.91)
|
0.01
|
|
CXCR4/CD26 ratio on naive T-helper lymphocytes
|
2.34 (0.87–5.68)
|
1.32 (0.51–3.01)
|
0.0002
|
|
CXCR4/CD26 ratio on memory T-helper lymphocytes
|
1.38 (0.79–2.82)
|
1.39 (0.71–2.50)
|
0.25
|
|
CXCR4/CD26 ratio on activated T-helper lymphocytes
|
1.70 (0.70–2.96)
|
1.63 (0.99–2.65)
|
0.93
|
