Monocyte activation and acquired autoimmune protein S deficiency promote disseminated intravascular coagulation in a patient with primary antiphospholipid syndrome.

ABSTRACT: Autoimmune protein S (PS) deficiency is a highly thrombotic, potentially life-threatening disorder. Its pathophysiological relevance in the context of primary antiphospholipid syndrome (APS) is unclear. Here, we report the case of a 76-year-old woman, who presented with a painful reticular skin erythema caused by microvascular thromboses. Disseminated intravascular coagulation (DIC) with consumptive coagulopathy was controlled only by continuous anticoagulation. While significantly elevated IgM antibodies to cardiolipin and β₂-glycoprotein-I were consistent with primary APS, a function-blocking PS autoantibody of the IgG isotype was detected. Robust microvesicle (MV)-associated tissue factor (TF) procoagulant activity (PCA) was isolated from patient plasma. Moreover, patient IgG

SUBMITTER: Beckmann L 

PROVIDER: S-EPMC8265818 | biostudies-literature | 2021 Jul

REPOSITORIES: biostudies-literature