hematology and oncology
Antiphospholipid Syndrome (APS)
Catastrophic antiphospholipid syndrome
In a small proportion of patients with APS, widespread thromboses occur in small vessels supplying multiple organs, often including the brain (causing neurologic defects). This syndrome is called catastrophic APS (CAPS) and can be confused with Disseminated Intravascular Coagulation (DIC) (DIC), The diagnosis of CAPS should be considered in patients with multiorgan failure (dysfunction of three or more organs) who have positive laboratory test results for APS. Acute kidney injury, encephalopathy, adrenal hemorrhage (due to thrombosis), skin necrosis, and Diffuse Alveolar Hemorrhage are known manifestations. Treatment includes high-dose corticosteroids, anticoagulation, plasmapheresis, and sometimesrituximab(an anti-CD20 monoclonal antibody) oreculizumab(an anti-complement component C5 monoclonal antibody). 