Baseline characteristics of the complete cohort
| Characteristic . | Total (N = 62) . |
|---|---|
| Sex | |
| Male | 32 (51.6%) |
| Female | 30 (48.4%) |
| Age, median (range) | 71 (34-93) |
| Race | |
| Asian/Pacific Islander | 2 (3.2%) |
| Black | 7 (11.3%) |
| Native American | 1 (1.6%) |
| Other | 2 (3.2%) |
| White | 46 (74.2%) |
| Unknown | 4 (6.5%) |
| Ethnicity | |
| Hispanic/Latino | 4 (6.5%) |
| Not Hispanic/Latino | 58 (93.5%) |
| Comorbidities∗ | |
| Vascular disease | 33 (53.2%) |
| Metabolic syndrome | 25 (40.3%) |
| Autoimmune disease | 14 (22.6%) |
| Cancer | 11 (17.7%) |
| Chronic kidney disease | 10 (16.1%) |
| Pulmonary disease | 9 (14.5%) |
| Venous thromboembolism | 7 (11.3%) |
| Dementia | 4 (6.5%) |
| Infection | 3 (4.8%) |
| Postpartum | 3 (4.8%) |
| Recurrent pancreatitis | 2 (3.2%) |
| None | 9 (14.5%) |
| Relevant medications at diagnosis∗ | |
| Antiplatelet† | 11 (17.7%) |
| Therapeutic anticoagulation‡ | 10 (16.1%) |
| Immunosuppression | 8 (12.9%) |
| Reason for AHA evaluation∗ | |
| Abnormal bleeding | 61 (98.4%) |
| Laboratory findings | 34 (54.8%) |
| Laboratory results at diagnosis | |
| Lowest FVIII activity level <1%§ | 38 (61.3%) |
| Lowest FVIII activity level 1%-5%§ | 19 (30.6%) |
| Lowest FVIII activity level >5%§ | 5 (8.1%) |
| Maximum FVIII inhibitor titer (BU/mL), median (range) | 74 (0.8-1100) |
| Maximum porcine inhibitor titer (BU/mL), median, (range)|| | 3 (0-24) |
| Lowest hemoglobin (g/dL)¶ | 6.8 (3.6-12.5) |
| Characteristic . | Total (N = 62) . |
|---|---|
| Sex | |
| Male | 32 (51.6%) |
| Female | 30 (48.4%) |
| Age, median (range) | 71 (34-93) |
| Race | |
| Asian/Pacific Islander | 2 (3.2%) |
| Black | 7 (11.3%) |
| Native American | 1 (1.6%) |
| Other | 2 (3.2%) |
| White | 46 (74.2%) |
| Unknown | 4 (6.5%) |
| Ethnicity | |
| Hispanic/Latino | 4 (6.5%) |
| Not Hispanic/Latino | 58 (93.5%) |
| Comorbidities∗ | |
| Vascular disease | 33 (53.2%) |
| Metabolic syndrome | 25 (40.3%) |
| Autoimmune disease | 14 (22.6%) |
| Cancer | 11 (17.7%) |
| Chronic kidney disease | 10 (16.1%) |
| Pulmonary disease | 9 (14.5%) |
| Venous thromboembolism | 7 (11.3%) |
| Dementia | 4 (6.5%) |
| Infection | 3 (4.8%) |
| Postpartum | 3 (4.8%) |
| Recurrent pancreatitis | 2 (3.2%) |
| None | 9 (14.5%) |
| Relevant medications at diagnosis∗ | |
| Antiplatelet† | 11 (17.7%) |
| Therapeutic anticoagulation‡ | 10 (16.1%) |
| Immunosuppression | 8 (12.9%) |
| Reason for AHA evaluation∗ | |
| Abnormal bleeding | 61 (98.4%) |
| Laboratory findings | 34 (54.8%) |
| Laboratory results at diagnosis | |
| Lowest FVIII activity level <1%§ | 38 (61.3%) |
| Lowest FVIII activity level 1%-5%§ | 19 (30.6%) |
| Lowest FVIII activity level >5%§ | 5 (8.1%) |
| Maximum FVIII inhibitor titer (BU/mL), median (range) | 74 (0.8-1100) |
| Maximum porcine inhibitor titer (BU/mL), median, (range)|| | 3 (0-24) |
| Lowest hemoglobin (g/dL)¶ | 6.8 (3.6-12.5) |
Percentages do not add up to 100% because respondents could select multiple answers for this variable.
The following indications for antiplatelet therapy (some had multiple) were provided: cardiovascular disease (n = 7), atrial fibrillation (n = 2), and stroke (n = 2).
The following indications for anticoagulation (some had multiple) were provided: atrial fibrillation (n = 7), venous thrombosis (n = 3), and prophylactic heparin with hemodialysis (n = 1).
FVIII activity level was measured with either a 1-stage or chromogenic assay before starting emicizumab.
Porcine inhibiter titer was only available for 42 patients.
Hemoglobin level was only available for 55 patients.