Table 2.
The primary etiologies of Budd-Chiari syndrome (n = 94)
Etiology | n (%) | |
FVLM (64 tested) | Homozygous | 10 (15.6) |
Heterozygous | 24 (37.5) | |
MTHFR (60 tested) | Homozygous | 8 (13.3) |
Heterozygous | 23 (38.3) | |
PGM (60 tested) | Homozygous | 1 (1.7) |
Heterozygous | 2 (3.3) | |
JAK2 (MPD) (62 tested) | + | 18 (29) |
Primary APA | + | 16 (17) |
Secondary APA | + | 11 (11.7) |
Behçet’s disease | + | 12 (12.8) |
Protein C deficiency | + | 4 (4.3) |
Antithrombin III deficiency | + | 4 (4.3) |
Protein S deficiency | + | 1 (1.1) |
PNH | + | 2 (2.1) |
Hormonal therapy (58 females) | + | 9 (15.5) |
Pregnancy-related (58 females) | + | 10 (17.2) |
FVLM: Factor V Leiden mutation; MTHFR: Methylene tetrahydrofolate reductase; PGM: Prothrombin gene mutation; JAK2: Janus tyrosine kinase-2; MPD: Myeloproliferative disorder; APA: Antiphospholipid antibody syndrome; PNH: Paroxysmal nocturnal hemoglobinuria.