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Abstrakt
Life-Threatening Autoimmune Hemolytic Anemia Treated with Manual Whole Blood Exchange with Rapid Clinical Improvement
Laura Cooling, Grace Boxer and Richard Simon
Severe autoimmune hemolytic anemia, with high titer panagglutinins, can present with severe intra-and extravascular hemolysis, complicated by renal failure, volume overload, hypertriglyceridemia, pancreatitis and multiorgan failure. We present a case of life-threatening warm autoimmune hemolytic anemia in a 19 year old male, refractory to steroids and splenectomy, with ongoing severe hemolysis, in vivo and in vitro autoagglutination, worsening hypoxia, methemoglobinemia, renal insufficiency, and hypertriglyceridemia. The patient underwent a single manual whole blood exchange with reconstituted whole blood (50% final hematocrit). The post-exchange hemoglobin was 7.7 gm/dL, with complete resolution of RBC autoagglutination, increased platelet count (20% ) and marked decreases in plasma free hemoglobin (43%), methemoglobin (9%), percent FiO2 (40%), creatinine (30%), triglycerides (66%), bilirubin (56%) and autoantibody titer (1000 to 256). The patient was extubated shortly after WBEx with minimal hemolysis and RBC transfusion support over the next 2 weeks.