Table 2 Comparisons of clinical and biological characteristics (a) and lymphocyte parameters (b) between HHT patients with or without history of severe infection
HHT with HSI | HHT without HSI | p | |
|---|---|---|---|
(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 |