Intracranial Bleeding as Primary Manifestation of Antiphospholipid Syndrome: Case Report

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder usually resulting in an increased risk of blood clots in the arterial-, venous-, or microvascular system. Various antibodies have been identified causing the activation and aggregation of thrombocytes and endothelial dysfunction resulting in a wide range of symptoms. Frequent manifestation may be arterial or venous clots in extremities or internal organs, neurological manifestation or pregnancy morbidities. Rarely, APS can manifest in the form of hemorrhage. We discuss the case of a 40-year-old male presenting with a severe headache, fatigue and fever. Head CT confirmed a subdural hematoma. Laboratory findings showed anemia, thrombocytopenia and elongated coagulation times. Immunoserology found the strong presence of lupus anticoagulant, anti-cardiolipin-, and anti-β2glycoprotein-I IgG antibodies confirming the diagnosis of primary antiphospholipid syndrome, specifically LA - hypoprothrombinemia syndrome. Following the administration of high dose glucocorticoids, we chose to give intravenous immunoglobulin followed by rituximab. Although his symptoms regressed and there was no further hemorrhaging, we chose to perform plasmapheresis due to continuously high concentration of anti- phospholipid antibodies to lower the antibody titer, which proved to be effective. Antithrombotic treatment can be challenging in such cases due to the co-occurrence of hypoprothrombinemia and prothrombotic antibodies. We decided to administer low-dose aspirin, with which the patient has no bleeding or blood clots for over 6 years. We find the case worth presenting due to the rare occurance of bleeding as primary manifestation of APS and due to the challenging treatment.