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.

Budd‐chiari syndrome causing acute liver failure: A multicenter case series

“…We found 19 ALF‐BCS cases published among 17 patient reports/series that considered all forms of BCS . No patient series specifically focused on ALF‐BCS and all were published prior to 2007.…”

“…We found 19 ALF-BCS cases published among 17 patient reports/series that considered all forms of BCS. (10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23)(24)(25) No patient series specifically focused on ALF-BCS and all were published prior to 2007. The characteristics of the 19 patients from the literature are presented in Table 3.…”

Hematologic Abnormalities in the Antiphospholipid Syndrome

“…Moreover, activation of coagulation causes consumption of platelets and coagulation factors which contribute to bleeding. Disseminated intravascular coagulation has been rarely found in both primary and secondary APS [107,108], and some features of disseminated intravascular coagulation have been reported in 13%-15% of patients with CAPS [103,109]. The clinical and immunological characteristics seem to be similar in CAPS patients with and without the presence of disseminated intravascular coagulation features.…”