Contributions
Abstract: EP1321
Type: E-Poster Presentation
Session title: Thrombosis and vascular biology - Biology & Translational Research
Background
Currently, antiphospholipid syndrome (APS) is recognized as one of the most important causes of acquired immune thrombophilia. Vascular accidents associated with the presence of antiphospholipid antibodies contribute greatly to morbidity and mortality. In addition, it is expected that APS, as an autoimmune disease, can lead to the development of a number of severe complications not associated with the risk of thrombosis.
Aims
To assess the nature of non-thrombotic complications of antiphospholipid syndrome.
Methods
The study included 55 patients aged from 14 to 61 years diagnosed with APS. The average age of patients was 36 ± 7.92 years, women predominated - 85.45%. The follow-up period ranged from 6 months to 7 years. The diagnosis was verified in accordance with the Sydney classification criteria of APS (2006). Antibodies (AB) to cardiolipin and to β2-glycoprotein-1 (β2-GP-1) of the IgM and G classes were determined by enzyme-linked immunosorbent assay (ELISA). Plasma hemostasis indicators and lupus anticoagulant were determined using an ACL Elite pro coagulometer. In order to exclude congenital thrombophilia, the activity of antithrombin III, proteins C and S, and the level of homocysteine were assessed. Molecular genetic testing (by PCR) for the presence of thrombogenic mutations was also carried out.
Results
The primary APS was identified in 90.9%. Among the clinical manifestations, obstetric pathology prevailed - 60% of cases. 23.6% of patients were diagnosed with thrombosis of various localization was diagnosed in: thromboembolism of the pulmonary arteries - 30.8%; vein thrombosis of the lower extremities - 30.8%; thrombosis of atypical localization (transverse sinus of the brain, portal vein) - 7.7%; ischemic cerebral circulation disorder - 16.5% of cases. In 4 patients (7.3%), the APS manifested with immune thrombocytopenia. Antibodies to β2-GP-1 of Ig M class were detected more often (36.4%); in 16.3% - antibodies to cardiolipin of the Ig M class and to β2-GP-1 of the IgG class. Highly positive APS was diagnosed in 27% of patients (n = 15), 60% of them developed complications within 2 to 3 years of follow-up, mainly due to the autoimmune mechanism of APS development. The most severe of them are aplastic anemia and aseptic necrosis of the heads of the pelvic bones, diagnosed in one patient at the age of 32 years. In two cases, the course of APS was complicated by the renal-cutaneous form of hemorrhagic vasculitis with the development of glomerulonephritis. The most common complication (44%) was autoimmune coagulopathy with pronounced decrease in plasma coagulation factors FVIII and FIX due to the presence of inhibitors to them. In one case, autoimmune hemolytic anemia was diagnosed against the background of APS.
Conclusion
The course of APS can be clinically manifested not only by thrombotic, but also by severe hematological complications, such as aplastic anemia, autoimmune coagulopathy, hemorrhagic vasculitis, autoimmune hemolytic anemia, and immune thrombocytopenia. In this regard, the diagnosis of primary APS should be considered much wider than the range of acquired thrombophilia.
Keyword(s): Antiphospholipid syndrome, Aplastic anemia, Coagulopathy, Venous thrombosis
Abstract: EP1321
Type: E-Poster Presentation
Session title: Thrombosis and vascular biology - Biology & Translational Research
Background
Currently, antiphospholipid syndrome (APS) is recognized as one of the most important causes of acquired immune thrombophilia. Vascular accidents associated with the presence of antiphospholipid antibodies contribute greatly to morbidity and mortality. In addition, it is expected that APS, as an autoimmune disease, can lead to the development of a number of severe complications not associated with the risk of thrombosis.
Aims
To assess the nature of non-thrombotic complications of antiphospholipid syndrome.
Methods
The study included 55 patients aged from 14 to 61 years diagnosed with APS. The average age of patients was 36 ± 7.92 years, women predominated - 85.45%. The follow-up period ranged from 6 months to 7 years. The diagnosis was verified in accordance with the Sydney classification criteria of APS (2006). Antibodies (AB) to cardiolipin and to β2-glycoprotein-1 (β2-GP-1) of the IgM and G classes were determined by enzyme-linked immunosorbent assay (ELISA). Plasma hemostasis indicators and lupus anticoagulant were determined using an ACL Elite pro coagulometer. In order to exclude congenital thrombophilia, the activity of antithrombin III, proteins C and S, and the level of homocysteine were assessed. Molecular genetic testing (by PCR) for the presence of thrombogenic mutations was also carried out.
Results
The primary APS was identified in 90.9%. Among the clinical manifestations, obstetric pathology prevailed - 60% of cases. 23.6% of patients were diagnosed with thrombosis of various localization was diagnosed in: thromboembolism of the pulmonary arteries - 30.8%; vein thrombosis of the lower extremities - 30.8%; thrombosis of atypical localization (transverse sinus of the brain, portal vein) - 7.7%; ischemic cerebral circulation disorder - 16.5% of cases. In 4 patients (7.3%), the APS manifested with immune thrombocytopenia. Antibodies to β2-GP-1 of Ig M class were detected more often (36.4%); in 16.3% - antibodies to cardiolipin of the Ig M class and to β2-GP-1 of the IgG class. Highly positive APS was diagnosed in 27% of patients (n = 15), 60% of them developed complications within 2 to 3 years of follow-up, mainly due to the autoimmune mechanism of APS development. The most severe of them are aplastic anemia and aseptic necrosis of the heads of the pelvic bones, diagnosed in one patient at the age of 32 years. In two cases, the course of APS was complicated by the renal-cutaneous form of hemorrhagic vasculitis with the development of glomerulonephritis. The most common complication (44%) was autoimmune coagulopathy with pronounced decrease in plasma coagulation factors FVIII and FIX due to the presence of inhibitors to them. In one case, autoimmune hemolytic anemia was diagnosed against the background of APS.
Conclusion
The course of APS can be clinically manifested not only by thrombotic, but also by severe hematological complications, such as aplastic anemia, autoimmune coagulopathy, hemorrhagic vasculitis, autoimmune hemolytic anemia, and immune thrombocytopenia. In this regard, the diagnosis of primary APS should be considered much wider than the range of acquired thrombophilia.
Keyword(s): Antiphospholipid syndrome, Aplastic anemia, Coagulopathy, Venous thrombosis