Catastrophic Antiphospholipid Syndrome after Orthotopic Liver Transplant. (5th May 2022)

• Record Type: Journal Article
• Authors: Okeke, R.; Lok, J.; Wells, R.; Wycoff, M.; Engelhardt, A.; Bettag, J.; O'Leary, C.; Hallcox, T.; Nazzal, M.
• Other Names: Grenda Ryszard Academic Editor.
• Is Part Of: Case reports in transplantation. Volume 2022(2022)
• Publication Date: 2022-05-05
• Subjects: Transplantation of organs, tissues, etc -- Periodicals; Transplantation; Transplantation of organs, tissues, etc; Periodicals; Periodicals; 617.954
• DOI: 10.1155/2022/6209300
• Languages: English
• ISSNs: 2090-6943

Abstract

Abstract : Background . Catastrophic antiphospholipid syndrome (CAPS) is an autoimmune thrombogenic disorder of small and large vessels caused by autoantibodies against phospholipids and phospholipid-binding proteins. This severe form of antiphospholipid syndrome (APS) presents clinically with simultaneous life-threatening multiorgan thrombosis and the presence of two or more persistent antiphospholipid antibodies (APL) confirmed on testing 12 weeks apart. Case Presentation . We describe a case report of a 66-year-old woman with detected antinuclear antibodies (ANA) pretransplant diagnosed with CAPS following orthotopic liver transplant. The patient had acute respiratory failure; Doppler ultrasound and CT angiogram confirmed thrombosis in the hepatic artery, subsequent occlusion of the jump graft, and a splenic infarct. Hypercoagulability workup showed elevated levels of anticardiolipin IgG and beta-2-glycoprotein IgG/IgM and positive lupus anticoagulant, treated with steroids and anticoagulation. The patient was discharged after one month and was transitioned from heparin to life-long warfarin. Conclusion . Our patient provided a standard presentation of CAPS with abnormal pretransplant levels of antinuclear antibodies (ANA). Although there have been studies investigating the relationship between anticardiolipin antibodies and lupus anticoagulants and APS, the relationship between pretransplant positive ANA or antimitochondrial antibodies (AMA) and CAPS has yet to be explored. Further studies will be needed to determine the significance of these antibodies. We recommend preoperative APL testing for patients with positive ANA and AMA at preliver transplant presentation.