Is porto sinusoidal vascular disease to be actively searched in patients with portal vein thrombosis?

Table 1 Diseases associated to porto sinusoidal vascular liver disease

Thrombophilia

Myeloproliferative neoplasm

Protein S or C deficiency

Antiphospholipid antibodies

Lupus anticoagulant

Factor V Leiden

Prothrombin mutation

Hematologic disease

Myeloproliferative neoplasm (polycythemia vera, chronic myelogenous leukaemia, essential thrombocythemia)

Myeloid metaplasia

Lymphoproliferative conditions (Hodgkin’s disease, non-Hodgkin’s lymphoma, chronic lymphocytic leukaemia and multiple myeloma)

Spherocytosis

Genetic disorders

Cystic fibrosis

Adams Oliver syndrome

Turner‘s disease

Autoimmune disease

Rheumatoid arthritis

Systemic lupus erythematosus

Systemic sclerosis

Scleroderma

Gut diseases

Celiac disease

Inflammatory bowel disease

Drug and toxics

Oxaliplatin

Azathioprine

6-thioguanine

Arsenic

Busulfan

Cytosine arabinoside

Cyclophosphamide

Bleomycin

Chlorambucil

Doxyrubicin

Carmustine

Acquired and congenital immunodeficiency

Human immunodeficiency virus

Primary antibody-deficiency syndrome

Table 2 Predisposing causes of portal vein thrombosis

Systemic prothrombotic conditions	Recommended checks
Inherited	Resistance to activated C protein/molecular biology for
Factor V Leiden	G1691A polymorphism
Prothrombin gene mutation	Molecular biology for G20210A polymorphism
Protein C, protein S and antithrombin deficiency	Ratio with F II, V, VII or X after correction for vitamin K deficiency; family survey (recommended)
Acquired
Antiphospholipid syndrome	Anticardiolipin ELISA; LLAC
Myeloproliferative neoplasm (MPN)	JAK2 and CALR mutations; osteomidullary biopsy
Paroxysomal nocturnal hemoglobinuria	Flow cytometry (deficient cells of CD55 and CD59)
Oral contraceptive use	Anamnesis
Obesity	BMI > 30 kg/m2
CMV infection	CMV IgG, CMV IgM
Pregnancy	Beta-HCG, anamnesis
Celiac disease	Anti-transglutaminase IgA/IgG
Local prothrombotic conditions	Anamnesis and radiological examination
Surgery and trauma
Umbilical cannulation
Splenectomy, cholecystectomy
Hepatic resection
Abdominal trauma
Bariatric surgery
Portosystemic shunts including TIPS	Anamnesis and radiological examination
Inflammatory and infectious diseases
Neonatal omphalitis
Appendicitis, diverticulitis, pancreatitis
Inflammatory bowel disease
Cholecystitis, cholangitis
Other
Cirrhosis
Porto sinusoidal vascular liver disease

LLAC: Lupus-like anticoagulant; ELISA: Enzyme-linked immunosorbent assay; BMI: Body mass index; CMV, Cytomegalovirus; HCG: Human chorionic gonadotropin; Ig: Immunoglobulin; TIPS: Transjugular portosystemic intrahepatic shunt.