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The differential diagnosis of splachnic vein thrombosis

It was with great interest that we read the review regarding splachnic vein thrombosis in myeloproliferative neoplasms (Sekhar et al, 2013). We were surprised by the fact that screening for two important diseases predisposing to a splachnic vein thrombosis (SVT), namely antiphospholipid syndrome (APS) and paroxysmal nocturnal haemoglobinuria (PNH), was not mentioned in the ‘algorithm for the management of portal vein thrombosis/Budd Chiari syndrome’, which does not refer exclusively to patients with myeloproliferative neoplasms. In this algorithm, F5 R506Q (Factor V Leiden, FVL) and F2 G20210A (prothrombin G 20210A) mutation screening is detailed. We are not clear why the authors do not mention other inherited thrombophilias, such as antithrombin, Protein C and Protein S deficiency alongside the two aforementioned as potential risk factors for SVT. However, as many guidelines recommend anticoagulation with a vitamin K antagonist after SVT, to prevent further thrombotic events (De Stefano & Martinelli, 2010), the rational for screening for inherited thrombophilias is poor because there are no studies showing that such practice changes clinical management. The British Society for Haematology guidelines on thrombophilia testing state that ‘testing for heritable thrombophilias after a first episode of intraabdominal vein thrombosis has uncertain predictive value for recurrence (C). Decisions regarding duration of anticoagulant therapy in relation to the results of testing are not evidence-based’ (Baglin et al, 2010).