Systemic congenital and acquired thrombophilia, such as Factor V Leiden, antithrombin III deficiency, and factor II G20210A mutation, as well as PNH should be considered within the differential diagnosis; splanchnic thrombosis has been reported to present in 7% of PNH cases in a recently published large cohort [15]. The gene discussed is SERPINC1; the disease is paroxysmal nocturnal hemoglobinuria.