Comparison of patients with hTTP described in case reports to patients with hTTP reported by the United Kingdom and International Hereditary TTP Registries
| . | hTTP . | |||||
|---|---|---|---|---|---|---|
| United Kingdom Registry10 . | International Registry11 . | Case reports patients . | ||||
| No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | Median (range) . | |
| Year of publication | 2019 | 2019 | 2022 | |||
| Patients | 73 | 120 | 226 | |||
| Female | 51 (70) | 62 (52) | 129 (57) | |||
| Age, y* | ||||||
| At diagnosis | 24 (newborn-71) | 17 (newborn-70) | 16 (newborn-77) | |||
| At last follow-up | NA | 26 (4-60) | 23 (0 d-79) | |||
| At first symptoms | 18 (newborn-67) | 5 (newborn-70) | 2 (newborn-63) | |||
| First symptoms at birth | NA | 30 (25) | 81 (36) | |||
| Stroke | 18 (25) | 37 (31) | 62 (27) | |||
| Age at stroke | NA | NA | 22 (1 d-77) | |||
| Myocardial infarction | 0 | 5 (4) | 5 (2) | |||
| Age at myocardial infarction | NA | NA | 20 (3-53) | |||
| Death | 5 (7) | NA | 11 (5)† | |||
| Age at death | NA | NA | 7 (newborn-79) | |||
| . | hTTP . | |||||
|---|---|---|---|---|---|---|
| United Kingdom Registry10 . | International Registry11 . | Case reports patients . | ||||
| No. (%) . | Median (range) . | No. (%) . | Median (range) . | No. (%) . | Median (range) . | |
| Year of publication | 2019 | 2019 | 2022 | |||
| Patients | 73 | 120 | 226 | |||
| Female | 51 (70) | 62 (52) | 129 (57) | |||
| Age, y* | ||||||
| At diagnosis | 24 (newborn-71) | 17 (newborn-70) | 16 (newborn-77) | |||
| At last follow-up | NA | 26 (4-60) | 23 (0 d-79) | |||
| At first symptoms | 18 (newborn-67) | 5 (newborn-70) | 2 (newborn-63) | |||
| First symptoms at birth | NA | 30 (25) | 81 (36) | |||
| Stroke | 18 (25) | 37 (31) | 62 (27) | |||
| Age at stroke | NA | NA | 22 (1 d-77) | |||
| Myocardial infarction | 0 | 5 (4) | 5 (2) | |||
| Age at myocardial infarction | NA | NA | 20 (3-53) | |||
| Death | 5 (7) | NA | 11 (5)† | |||
| Age at death | NA | NA | 7 (newborn-79) | |||
Sixty-eight (30%) of the case report patients were included in the International Registry.11 There were no case report patients included in the United Kingdom Registry.10
NA, not available.
Age is given in years, unless otherwise specified.
Case report data include 21 patients who died but who would not have been enrolled in the Registries: 18 patients who died without ADAMTS13 sequence analysis and 3 patients who had ADAMTS13 sequence confirmation but who died in their first days of life. Including these 21 deaths, the frequency of death would be 14%.