Table 4.
Patient | Agea | Sex | Number of TMA episodes prior to eculizumab | Family history of aHUS | Genetic variantb/antibody | Total days of eculizumab treatment | Recurrence of aHUS | Days after discontinuation | Treatment at last observation |
---|---|---|---|---|---|---|---|---|---|
1 | 10 years | F | 1 | Y | CFHR1 p.Asp35fsX36, anti-CFH antibody | 1010 | N | 337 | – |
2 | 10 years | M | 1 | N | CFHR3 exon 6 deletion, anti-CFH antibody | 105 | N | 1681 | PSL, CsA |
3 | 17 years | M | 2 | Y | C3 p.Ile1157Thr | 424 | N | 308 | – |
4 | 17 years | M | 3 | N | MCP p.Asp170Met FsX9 (Homo) | 1 | N | 408 | – |
5 | 5 years | M | 1 | N | Not examined, anti-CFH antibody | 357 | N | 1438 | – |
6 | 16 years | F | 1 | N | Not examined | 213 | N | 754 | – |
7 | 16 years | M | 1 | N | Not examined (positive for haemolytic assay) | 575 | N | 126 | Peritoneal dialysis |
8 | 7 years | M | 1 | N | CFHR1-CFHR3 deletion, anti-CFH antibody | 595 | N | 371 | – |
9 | 11 years | F | 1 | N | Not identified, anti-CFH antibody | 603 | N | 128 | – |
10 | 7 months | F | 1 | N | Not identified | 8 | N | 727 | PSL |
11 | 9 months | M | 1 | N | C3 p.Ser179Pro, CFH p.Hus402Tyr (homo), p.Glu936Asp (homo) | 327 | N | 465 | – |
12 | 4 years | M | 1 | N | CFHR1-CFHR3 deletion, anti-CFH antibody | 385 | N | 708 | PSL, MMF |
13 | 6 years | F | 1 | N | CFH p.His402Tyr, p.Glu936Asp, MCP p.Thr163Ile | 29 | N | 326 | – |
F, female; M, male; Y, yes; N, no; PSL, prednisolone; CsA, cyclosporin.
aAge at first administration. bThe information on homozygosity or heterozygosity of the genetic variant is not available if there is no description in this table.