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.