Table I.
Patient Group | Patients treated with C5 inhibitor monotherapy (n=18) | Patients treated with C5 inhibitor and indefinite anticoagulation (n=4) |
---|---|---|
| ||
History of aplastic anemia, no. | 7 | 0 |
| ||
Sex, no. | ||
Male | 11 | 1 |
Female | 7 | 3 |
| ||
Race | ||
Caucasian | 14 | 1 |
Black | 4 | 3 |
| ||
Median age at PNH diagnosis (range) | 23.5 years (10–50) | 41 years (36–61) |
| ||
Median PNH granulocyte clone at TE diagnosis (range) | 96% (73–100) | 87.8% (78–99) |
| ||
Median disease duration pre-C5 inhibition (range) | 4 years (0–14) | 3.5 years (0.5–8) |
| ||
Median time on anti-C5 treatment (range) | 10 years (0.5–15) | 9.5 years (5–13) |
| ||
Median time on anticoagulation (range) | 4 months (0–11 years) | 9 years (4–19) |
| ||
Location of TE events prior to C5 inhibition, no. † | ||
DVT | 1 | 1 |
pulmonary embolism | 1 | 1 |
abdominal vein | 11 | 2 |
dermal | 1 | - |
small bowel | 3 | - |
cerebrovascular | 3 | 2 |
IVC | 1 | - |
renal vein | 2 | - |
ureter | 1 | - |
tonsillar | 1 | - |
| ||
Location of TE events on C5 inhibitor, no. | ||
DVT | 2‡ | 1§ |
pulmonary embolism | - | 1 |
28/31 TE events occurred not on anticoagulation.
Not on concurrent anticoagulation. Two lower extremity deep vein thromboses (DVT) around major surgeries, hip replacement and liver transplant, respectively.
1 bilateral DVT attributed to uterine compression.