Antiphosphatidylserine Antibody as a Cause of Multiple Dural Venous Sinus Thromboses and ST-Elevation Myocardial Infarction

Abstract

Patient: Male, 48Final Diagnosis: Antiphospholipid syndromeSymptoms: Chest pain • confusion • seizure-like activityMedication: —Clinical Procedure: Endovascular venous suction thrombectomySpecialty: HematologyObjective:Rare diseaseBackground:Antiphospholipid syndrome (APS) is an autoimmune disease characterized by antibodies directed against phospholipids on plasma membranes. Through unclear mechanisms, APS confers hypercoagulability. APS may cause recurrent thromboses in the arterial and venous vasculature. We report a case of primary APS resulting in cerebral venous thrombosis and ST-elevation myocardial infarction (STEMI) for which only antiphosphatidylserine (aPS) IgM antibody was positive after extensive investigation.Case Report:A 48-year-old male was admitted after a witnessed generalized seizure with subsequent confusion. Imaging demonstrated thrombosis of multiple central nervous system (CNS) sinuses, including the superior sagittal sinus and bilateral transverse sinuses. The patient was heparinized with aggressive hydration, which proved inadequate, prompting endovascular thrombectomy. Three months later, despite anticoagulation therapy, the patient developed a STEMI when International Normalized Ratio (INR) was 1.8. Echocardiogram (ECHO) and PAN CT scan were normal. Initial coagulation studies demonstrated normal anticardiolipin antibody, prothrombin time, partial thromboplastin time, and platelet count. Outpatient coagulation studies revealed normal antithrombin III, protein C/S, hemoglobin electrophoresis, homocysteine, anti-β2 glycoprotein 1 antibodies, and D-Dimer. Factor V Leiden, JAK 2 mutation, prothrombin gene mutation, and tests for paroxysmal nocturnal hemoglobinuria (PNH) were negative. A positive phosphatidylserine IgM was detected. The patient was continued on warfarin (10 mg daily) with a target INR of 3.0–3.5 and clopidogrel (75 mg daily).Conclusions:Despite extensive investigation, this patient only showed evidence of elevated aPS IgM antibodies, likely contributing to his CNS venous sinus thromboses and STEMI. It is important to screen for antiphosphatidylserine antibodies in cases of unprovoked thrombosis when standard thrombophilia analysis is unrevealing. This will assist in identifying pathogenicity and help prevent recurrence of subsequent thromboses.

Particularities of Acute Myocardial Infarction in Young Adults

“…31 Primary or secondary thrombocytosis may represent a cause of myocardial infarction in young people and have been described as precipitating factors for coronary artery occlusion in case of pre-existing atheromatous lesions, although myocardial infarction caused by splenectomy secondary to thrombocytosis has been reported in the absence of coronary changes suggestive of atherothrombotic lesions. 32 Also, mutations and dysfunctions of factor V Leiden are associated with a high risk of cardiovascular events, being more commonly reported in smokers and in those who use oral contraceptives, because they increase the risk through their procoagulant effect. 33,34 Connective tissue diseases, through chronic systemic inflammation, and sometimes through long-term therapy, favor coronary atherosclerosis.…”