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Recurrent disseminated intravascular coagulation and fulminant intra hepatic thrombosis in a patient with the anti‐phospholipid syndrome
Author(s) -
Stinson J.,
Tomkin G.,
McDonald G.,
Jackson F.,
Harrison M.,
Murray A.,
Feighery C.,
Jackson J.
Publication year - 1990
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830350413
Subject(s) - coagulopathy , medicine , disseminated intravascular coagulation , fulminant , fulminant hepatic failure , budd–chiari syndrome , lupus anticoagulant , gastroenterology , thrombosis , pathology , liver transplantation , transplantation , inferior vena cava
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.