Current concepts in the management of non-cirrhotic non-malignant portal vein thrombosis

Table 1 Risk factors for non-cirrhotic non-malignant portal vein thrombosis, %

Risk factor	Prevalence	Investigations
Systemic
Myeloproliferative neoplasm	21-32	JAK2 V617F mutation testing
		CALR mutation testing if platelets > 200 × 109/L and spleen ≥ 16 cm
		Consider bone marrow biopsy
Acquired thrombophilia
Antiphospholipid syndrome	6	Lupus anticoagulant, anti-cardiolipin and anti-b2 glycoprotein-I antibodies (2 positive samples 12 wk apart)
Paroxysmal nocturnal haemoglobinuria	0.30	Flow cytometry (CD55 and CD59 deficient cells)
Inherited thrombophilia
Prothrombin G20210A gene mutation	6-7	Prothrombin G20210A mutation testing
Factor V Leiden	3-7	Factor V Leiden mutation testing
Protein C deficiency	5-6	Protein C levels1
Protein S deficiency	3-5	Protein S levels1
Antithrombin deficiency	1-4	Antithrombin levels1
Hormonal (recent pregnancy/oral contraceptive)	16	Medical history
Other systemic disease e.g., connective tissue disease, sarcoidosis, vasculitis, acute CMV infection	3	Variable
Obesity		Medical history
Local
Abdominal trauma/surgery	14	Medical history/cross-sectional imaging
Inflammatory abdominal conditions e.g., pancreatitis, biliary infection, appendicitis, inflammatory bowel disease	11	Variable
No cause identified	35-42

¹Interpret with caution in liver impairment.

Risk factors for non-cirrhotic non-malignant portal vein thrombosis[1,19]. CMV: Cytomegalovirus; CALR: Calreticulin.