Main features of the AHASR cohort patients
| Variable . | n/N (%)* . |
|---|---|
| Age at DX (y), median (IQR) | 74 (64-83) |
| Male | 85/151 (56.3) |
| Follow-up (mo)†, median (IQR) | |
| Whole cohort (n = 154) | 12 (2-33) |
| Patients alive at study end (n = 36) | 15 (4-36) |
| FVIII:C at Dx, median (IQR) | 1.9 (0.6-4.7) |
| 6-40 IU/dL | 27/135 (20.0) |
| 1-5 IU/dL | 70/135 (51.8) |
| <1 IU/dL | 38/135 (28.1) |
| Inhibitor at Dx, median (IQR) | 16.00 (5.50-42.40) |
| 0-10 BU/mL | 54/145 (37.2) |
| >10-100 BU/mL | 76/145 (52.4) |
| 101-1000 BU/mL | 11/145 (7.6) |
| >1000 BU/mL | 4/145 (2.8) |
| Bleeding at Dx‡ | 135/149 (90.6) |
| Mucocutaneous | 91/132 (68.9) |
| Muscular | 41/132 (31.1) |
| Urinary | 22/132 (16.7) |
| Digestive | 16/132 (12.1) |
| Retroperitoneal | 13/132 (9.8) |
| Thoracic | 13/132 (9.8) |
| Other§ | 16/132 (12.1) |
| Required bypassing treatment | 67/95 (70.5) |
| rFVIIa | 38/63 (60.3) |
| aPCC | 13/63 (20.6) |
| Both | 12/63 (19.0) |
| Underlying disease/condition║ | |
| Idiopathic | 64/145 (44.1) |
| Autoimmune disorder¶ | 46/145 (31.7) |
| Type 1 diabetes | 5/46 (10.9) |
| Rheumatoid arthritis | 4/46 (8.7) |
| Rheumatic polymyalgia | 5/46 (10.9) |
| Other/nonspecified** | 21/46 (45.6) |
| Neoplasia | 15/145 (10.3) |
| Colon | 4/15 (26.7) |
| Prostate | 3/15 (20.0) |
| Other†† | 7/15 (46.7) |
| Postpartum | 9/145 (6.2) |
| Other | 19/145 (13.1) |
| Received IST | 142/147 (96.6) |
| Achieved CR | 112/133 (84.2) |
| Relapsed after CR | 8/112 (7.1) |
| Time to relapse (d), median (IQR) | 178 (125-248) |
| Variable . | n/N (%)* . |
|---|---|
| Age at DX (y), median (IQR) | 74 (64-83) |
| Male | 85/151 (56.3) |
| Follow-up (mo)†, median (IQR) | |
| Whole cohort (n = 154) | 12 (2-33) |
| Patients alive at study end (n = 36) | 15 (4-36) |
| FVIII:C at Dx, median (IQR) | 1.9 (0.6-4.7) |
| 6-40 IU/dL | 27/135 (20.0) |
| 1-5 IU/dL | 70/135 (51.8) |
| <1 IU/dL | 38/135 (28.1) |
| Inhibitor at Dx, median (IQR) | 16.00 (5.50-42.40) |
| 0-10 BU/mL | 54/145 (37.2) |
| >10-100 BU/mL | 76/145 (52.4) |
| 101-1000 BU/mL | 11/145 (7.6) |
| >1000 BU/mL | 4/145 (2.8) |
| Bleeding at Dx‡ | 135/149 (90.6) |
| Mucocutaneous | 91/132 (68.9) |
| Muscular | 41/132 (31.1) |
| Urinary | 22/132 (16.7) |
| Digestive | 16/132 (12.1) |
| Retroperitoneal | 13/132 (9.8) |
| Thoracic | 13/132 (9.8) |
| Other§ | 16/132 (12.1) |
| Required bypassing treatment | 67/95 (70.5) |
| rFVIIa | 38/63 (60.3) |
| aPCC | 13/63 (20.6) |
| Both | 12/63 (19.0) |
| Underlying disease/condition║ | |
| Idiopathic | 64/145 (44.1) |
| Autoimmune disorder¶ | 46/145 (31.7) |
| Type 1 diabetes | 5/46 (10.9) |
| Rheumatoid arthritis | 4/46 (8.7) |
| Rheumatic polymyalgia | 5/46 (10.9) |
| Other/nonspecified** | 21/46 (45.6) |
| Neoplasia | 15/145 (10.3) |
| Colon | 4/15 (26.7) |
| Prostate | 3/15 (20.0) |
| Other†† | 7/15 (46.7) |
| Postpartum | 9/145 (6.2) |
| Other | 19/145 (13.1) |
| Received IST | 142/147 (96.6) |
| Achieved CR | 112/133 (84.2) |
| Relapsed after CR | 8/112 (7.1) |
| Time to relapse (d), median (IQR) | 178 (125-248) |
One hundred and fifty-four patients were recruited for the AHASR until September 2020. Data corresponding to the moment of the AHA diagnosis are depicted. Missing data were not considered.
Dx, diagnosis; LAC, lupus anticoagulant.
Unless otherwise specified.
For patients who were alive, the time between the date of diagnosis and the date of the last contact (by any means) was considered to calculate the follow-up period. For those who died, the time between diagnosis and exitus was calculated.
Fifty-six patients had bleeding in more than 1 site.
Postpartum bleeding (3), retropharyngeal (2), hemarthros (3), hemopericardium (1), gynecological bleeding not related to pregnancy/postpartum (4), breast bleeding (1), carotid hematoma subsequent to lymph node biopsy (1), postpuncture upper limb hematoma (1).
Seven patients had more than one underlying disease/condition.
Twelve patients had LAC.
Pemphigus (2), bowel inflammatory disease (1), psoriatic arthritis (1), anti-nuclear antibodies (2), nonspecified (15).
Bladder (2), kidney (1), multiple myeloma (1), chronic lymphocytic leukemia (1), lung (1), nonspecified (2).