2019 Volume 56 Issue 2 Pages 238-242
A 15-year-old girl with catastrophic antiphospholipid syndrome (CAPS) presented with severe thrombocytopenia and hemolytic anemia and became brain dead on the fourth day of hospitalization because of hemorrhagic stroke. She visited the hospital because of fever and malaise. She had thrombocytopenia (Plt 8,000/μL) and hemolytic anemia (Hb 3.2 g/dL). She was refractory to various treatments including blood transfusions, intravenous methylprednisolone, plasma exchange, multiple doses of intravenous immunoglobulin infusion, and percutaneous splenic artery obliteration. Despite these treatments, her platelets were less than 10,000/μL, and she had hemorrhagic stroke. Progressing cerebral hemorrhage led to brain death. CAPS is one of the most important diseases when we examine a patient who has thrombophilia. This case illustrates that it is difficult to diagnose CAPS early; despite its acute fulminant course, initial treatments with CAPS requires assessments and treatments many times. Physicians must keep this possibility in mind.