title: Recurrent disseminated intravascular coagulation and fulminant intra hepatic thrombosis in a patient with the anti-phospholipid syndrome.
abstract: We describe a patient with the lupus anti-coagulant who had recurrent episodes, over a 2 year period, of a severe and disseminated intravascular coagulopathy. This patient also had positive serological assays for syphilis and anti-cardiolipin antibodies. Associated with the coagulopathy were co-expressed episodes of liver disease, ultimately terminating in fulminant liver failure. At autopsy the features were characteristic of the Budd-Chiari syndrome. This is the first report to document how consumptive coagulopathy may present as a dominant feature of the anti-phospholipid syndrome. It also clearly describes an immune mediated thrombotic mechanism as a cause of hepatic veno-occlusive disorders. Furthermore, this case highlights the varied clinical spectrum of the anti-phospholipid syndrome and suggests that a high index of suspicion is required to ensure its diagnosis.
authors.affiliation: Adelaide Hospital, Dublin, Ireland.
authors.forename: J
authors.initials: J
authors.lastname: Stinson
authors.forename: G
authors.initials: G
authors.lastname: Tomkin
authors.forename: G
authors.initials: G
authors.lastname: McDonald
authors.forename: F
authors.initials: F
authors.lastname: Jackson
authors.forename: M
authors.initials: M
authors.lastname: Harrison
authors.forename: A
authors.initials: A
authors.lastname: Murray
authors.forename: C
authors.initials: C
authors.lastname: Feighery
authors.forename: J
authors.initials: J
authors.lastname: Jackson
mesh_terms: D000328:Adult; D000906:Antibodies / Q000032:analysis; D001327:Autoimmune Diseases / Q000150:complications* / Q000175:diagnosis; D006502:Budd-Chiari Syndrome / Q000150:complications*; D004211:Disseminated Intravascular Coagulation / Q000150:complications*; D005260:Female; D006801:Humans; D010743:Phospholipids / Q000276:immunology*; D013577:Syndrome
chemical_list: D000906:Antibodies; D010743:Phospholipids