Recurrent disseminated intravascular coagulation and fulminant intra hepatic thrombosis in a patient with the anti-phospholipid syndrome.
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.
Stinson
J
J
Adelaide Hospital, Dublin, Ireland.
Tomkin
G
G
McDonald
G
G
Jackson
F
F
Harrison
M
M
Murray
A
A
Feighery
C
C
Jackson
J
J
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
D000906:Antibodies; D010743:Phospholipids