Health outcomes of 226 patients with hTTP
| . | No. (%) . | Median (range) . |
|---|---|---|
| All patients | 226 | |
| Females | 129 (57) | |
| Patients with symptoms of TTP* | 216 | |
| Females | 121 (56) | |
| Patients with age at first symptoms reported | 204 | |
| Females | 114 (56) | |
| Patients with age at diagnosis reported | 202 | |
| Females | 117 (58) | |
| Age, y† | ||
| At first symptoms | 2 (0-63) | |
| At diagnosis | 16 (0-77) | |
| At death‡ | 32 (14) | |
| Neonatal | 9 | 0-17 d |
| Pregnancy | 4 | 23 (20-26) |
| Children | 9 | 5 (2-13) |
| Adults | 10 | 40 (21-79) |
| Last follow-up§ | 23 (0-79) | |
| Major morbidities | ||
| Patientsǁ | 73 (34) | |
| Age at initial morbidity | 21 y (1 d-77 y) | |
| Females | 38 (52) | |
| Types of major morbidities¶ | ||
| Stroke | 62 (27) | |
| Kidney injury | 23 (10) | |
| Cardiac injury | 8 (4) |
| . | No. (%) . | Median (range) . |
|---|---|---|
| All patients | 226 | |
| Females | 129 (57) | |
| Patients with symptoms of TTP* | 216 | |
| Females | 121 (56) | |
| Patients with age at first symptoms reported | 204 | |
| Females | 114 (56) | |
| Patients with age at diagnosis reported | 202 | |
| Females | 117 (58) | |
| Age, y† | ||
| At first symptoms | 2 (0-63) | |
| At diagnosis | 16 (0-77) | |
| At death‡ | 32 (14) | |
| Neonatal | 9 | 0-17 d |
| Pregnancy | 4 | 23 (20-26) |
| Children | 9 | 5 (2-13) |
| Adults | 10 | 40 (21-79) |
| Last follow-up§ | 23 (0-79) | |
| Major morbidities | ||
| Patientsǁ | 73 (34) | |
| Age at initial morbidity | 21 y (1 d-77 y) | |
| Females | 38 (52) | |
| Types of major morbidities¶ | ||
| Stroke | 62 (27) | |
| Kidney injury | 23 (10) | |
| Cardiac injury | 8 (4) |
Ten patients who had no symptoms of TTP were diagnosed with hTTP by documentation of biallelic ADAMTS13 mutations (described in supplemental Table 3).
Units for age are years, unless otherwise indicated.
Nine patients died during their first days of life. When these neonatal deaths were excluded, the frequency of death was 11%, the youngest age was 2 years, and the median age was 22 years.
Age at last follow-up was not reported for 5 of the 194 surviving patients.
Percent of 217 patients, omitting the 9 infants who died at birth.
Eighteen of the 73 patients initially had 2 of the 3 categories of morbidities; 1 patient initially had all 3 morbidities. Cardiac injuries were myocardial infarction (5 patients), cardiomyopathy (2 patients), and congestive heart failure (1 patient).