Clinical characteristics and prothrombotic factors in patients with Budd-Chiari syndrome and portal vein thrombosis
| . | BCS, n = 107 . | PVT, n = 92 . |
|---|---|---|
| Median age, y (Q1-Q3) | 38.1 (28-51) | 49.8 (42-57) |
| Males | 45 (42) | 43 (47) |
| Inherited thrombophilia* | 23 (21) | 22 (24) |
| Protein C deficiency | 2 (2) | 2 (2) |
| Protein S deficiency | 1 (1) | 5 (6) |
| Antithrombin deficiency | 3 (3) | 3 (4) |
| Factor V Leiden mutation | 12 (11) | 3 (3) |
| Prothrombin gene G20210A | 5 (5) | 12 (13) |
| Acquired thrombophilia* | 83 (78) | 56 (61) |
| Myeloproliferative neoplasms | 42 (39) | 24 (26) |
| Polycythemia vera | 24 (57) | 3 (13) |
| Essential thrombocytosis | 7 (17) | 10 (42) |
| Primary myelofibrosis | 2 (5) | 4 (17) |
| Unclassifiable | 9 (21) | 7 (29) |
| JAK2V617F-positive | 34 (32) | 20 (22) |
| History of myeloproliferative neoplasms | 10 (24) | 6 (25) |
| Antiphospholipid antibodies | 27 (25) | 25 (28) |
| Paroxysmal nocturnal hemoglobinuria | 12 (21) | 0 (0) |
| Hormonal | 23 (37) | 18 (37) |
| Systemic disorder† | 12 (11) | 3 (3) |
| Local risk factor‡ | 15 (14) | 25 (28) |
| Single risk factor§ | 49 (46) | 29 (32) |
| Multiple risk factors§ | 44 (41) | 43 (47) |
| No risk factor | 14 (9) | 20 (22) |
| . | BCS, n = 107 . | PVT, n = 92 . |
|---|---|---|
| Median age, y (Q1-Q3) | 38.1 (28-51) | 49.8 (42-57) |
| Males | 45 (42) | 43 (47) |
| Inherited thrombophilia* | 23 (21) | 22 (24) |
| Protein C deficiency | 2 (2) | 2 (2) |
| Protein S deficiency | 1 (1) | 5 (6) |
| Antithrombin deficiency | 3 (3) | 3 (4) |
| Factor V Leiden mutation | 12 (11) | 3 (3) |
| Prothrombin gene G20210A | 5 (5) | 12 (13) |
| Acquired thrombophilia* | 83 (78) | 56 (61) |
| Myeloproliferative neoplasms | 42 (39) | 24 (26) |
| Polycythemia vera | 24 (57) | 3 (13) |
| Essential thrombocytosis | 7 (17) | 10 (42) |
| Primary myelofibrosis | 2 (5) | 4 (17) |
| Unclassifiable | 9 (21) | 7 (29) |
| JAK2V617F-positive | 34 (32) | 20 (22) |
| History of myeloproliferative neoplasms | 10 (24) | 6 (25) |
| Antiphospholipid antibodies | 27 (25) | 25 (28) |
| Paroxysmal nocturnal hemoglobinuria | 12 (21) | 0 (0) |
| Hormonal | 23 (37) | 18 (37) |
| Systemic disorder† | 12 (11) | 3 (3) |
| Local risk factor‡ | 15 (14) | 25 (28) |
| Single risk factor§ | 49 (46) | 29 (32) |
| Multiple risk factors§ | 44 (41) | 43 (47) |
| No risk factor | 14 (9) | 20 (22) |
Values are n (%) unless otherwise specified.
BCS indicates Budd-Chiari syndrome; and PVT, portal vein thrombosis.
Patients can have more than one thrombophilic factor simultaneously. Not all investigations could be performed in the individual patients.
Behçet disease, sarcoidosis, vasculitis, or connective tissue disease.
Intra-abdominal inflammation, infection, or abscess.
Single risk factor: presence of an inherited or acquired thrombophilic factor or the presence of a local risk factor. Multiple risk factors: presence of 2 or more of these risk factors.