Table 2.
Data from the multicentric French cohort
| All patients | Patients with normal platelets count | Patients with thrombocytopenia | P | |
|---|---|---|---|---|
|
Number of patients |
107 |
14 |
93 |
|
|
Number of episodes |
145 |
17 |
128 |
|
|
Number of relapsing patients |
18 (20%) |
2 (14%) |
19 (20%) |
0.88 |
|
Sex (%male) |
29 (27%) |
5 (36%) |
24 (26%) |
0.39 |
|
Age (years) |
32 [22 – 45] |
32 [22 – 45] |
33 [23 – 42] |
0.78 |
|
Biopsy proven TMA |
72 (67%) |
13 (93%) |
59 (63%) |
|
|
identification of mutation |
75 (70%) |
10 (71%) |
65 (70%) |
0.95 |
| CFH |
40 |
6 |
34 |
|
| CFI |
17 |
3 |
14 |
|
| MCP |
10 |
1 |
9 |
|
| C3 |
11 |
1 |
10 |
|
| CFB |
|
|
2 |
|
| Anti-factor H Autoantibody |
|
|
2 |
|
|
Potential explanation |
2 (13.3%) |
1 (14.3%) |
11 (12%) |
|
| Familial form |
|
1 |
10 |
|
| Low C3 |
2 |
0 |
1 |
|
|
Idiopathic |
21 (23.3%) |
3 (21.4%) |
19 (25%) |
|
|
Pregnancy associated HUS |
19 (18%) |
1 (7%) |
17 (18%) |
0.151 |
|
Platelets (G/L)* |
79 [48-117] |
214 [186 - 242] |
68 [46-100] |
< 0.001 |
|
Hb (g/dl) |
7.2 [6,6 - 9] |
8.6 [6.7-10] |
7.2 [6.6 - 8.8] |
0.16 |
|
LDH* (UI/L) |
1688 [951-2930] |
664 [498-1914] |
1711 [999-1953] |
0.017 |
|
Creatinine (mg/dl) |
7.3 [4.1 - 11.3] |
4.3 [2.6 - 12] |
7.6 [4.6 - 11.3] |
0.27 |
|
HD at the diagnosis |
13 (12%) |
1 (7%) |
12 (13%) |
0.68 |
|
HD during the episode |
72 (80%) |
10 (71%) |
62 (81.6%) |
0.6 |
| Death Number/% | 6 (6.7%) | 1 (7.1%) | 5 (6.6%) | 0.9 |
For Numeric value, data are express in Median value [25%-75%], for categories, data are express in number of observation (%). Test used, Mann Whitney test (quantitative data) and Fisher's test for proportion and rate. Note, Conversion factors for units, serum creatinine in mg/dL to mol/L, ×88.4; serum urea nitrogen in mg/dL to mmol/L.