Recurrent disseminated intravascular coagulation and fulminant intra hepatic thrombosis in a patient with the anti-phospholipid syndrome

Autor: A. Murray, J. Jackson, Michael R. Harrison, C. Feighery, F. Jackson, G. McDonald, G. Tomkin, J. Stinson
Rok vydání: 1990
Předmět:
Zdroj: American Journal of Hematology. 35:281-282
ISSN: 1096-8652
0361-8609
DOI: 10.1002/ajh.2830350413
Popis: 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.
Databáze: OpenAIRE