An unusual case of epigastric pain

• Published in: BMJ (Online) Vol. 348; no. jun09 3; p. g3689
• Main Authors: Wijeyeratne, Yanushi Dullewe, Walters, Nicola
• Format: Journal Article
• Language: English
• Published: England British Medical Journal Publishing Group 09.06.2014; BMJ Publishing Group LTD
• Subjects: Abdomen; Abdominal Pain - etiology; Acute Disease; Humans; Liver; Male; Middle Aged; Mutation; Pain; PICTURE QUIZ; Portal Vein; Spleen; Veins & arteries; Venous Thrombosis - complications; Venous Thrombosis - diagnosis
• ISSN: 0959-8138; 1756-1833; 1756-1833
• DOI: 10.1136/bmj.g3689

Haematological disorders that may be considered include deficiencies of the coagulation factor inhibitors protein C and protein S, coagulation factor gene mutations, antithrombin deficiency, and the presence of antiphospholipid antibodies, including lupus anticoagulant. 14 Thrombophilia screening is controversial because it is costly and rarely influences the duration of anticoagulation. [...]chronic liver disease may result in reduced concentrations of protein C, protein S, and antithrombin. Paroxysmal nocturnal haemoglobinuria, where uncontrolled complement activity leads to systemic complications through intravascular haemolysis and platelet activation, can predispose to portal vein thrombosis and other thrombotic disorders. 15 Thrombotic disease may also be associated with myeloproliferative disease and may be the sole presenting feature. 16 It has been reported that 5-35% of patients with portal vein thrombosis may be positive for JAK2 V617F, a gain of function mutation in the JAK2 (janus kinase 2) gene that has been implicated in several myeloproliferative disorders. 17 18 JAK2 screening has been recommended for cases of unexplained or catastrophic portal vein thrombosis, given its high reported prevalence in this group of patients. 9 Liver function is usually preserved except in catastrophic portal vein thrombosis, probably because increased hepatic blood flow compensates for the portal venous obstruction. 9 In patients with cirrhosis, however, portal vein thrombosis can accelerate the progression of cirrhosis and make transplantation more difficult. 14 24 Undiagnosed chronic portal vein thrombosis leads to portal hypertension, and in developing countries it has been reported that 40% of portal hypertension may be caused by portal vein thrombosis. 9 Patient outcome Haematology advice was immediately sought after computed tomography and the patient was promptly started on anticoagulation with treatment-dose low molecular weight heparin followed by warfarin. [...]it was decided to continue the warfarin therapy, and this decision will be reviewed in six months' time.