title: A Fatal Case of Immune Hyperhemolysis with Bone Marrow Necrosis in a Patient with Sickle Cell Disease.
abstract: In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC) disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL) with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis. Despite treatment, the bone marrow failure did not improve and the patient died on hospital day 38. This case illustrates the potential risks of transfusion in a patient with sickle cell disease, especially one with previous hemolytic reactions. While uncommon, hyperhemolysis can cause death, in this case by extensive bone marrow necrosis. In patients with sickle cell disease, judicious use of red cell transfusions with phenotypically-matched units can diminish, but never completely abrogate, the risks associated with transfusion.
authors.affiliation: Medical Sciences Institute, Blood Center of Wisconsin, Milwaukee, WI; Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Matthew S
authors.initials: MS
authors.lastname: Karafin
authors.affiliation: Medical College of Wisconsin , Milwaukee, WI, USA.
authors.forename: Arun
authors.initials: A
authors.lastname: Singavi
authors.affiliation: Medical Sciences Institute , Blood Center of Wisconsin, Milwaukee, WI.
authors.forename: Susan T
authors.initials: ST
authors.lastname: Johnson
authors.affiliation: Medical Sciences Institute, Blood Center of Wisconsin, Milwaukee, WI; Medical College of Wisconsin, Milwaukee, WI, USA.
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Field
keywords: Bone marrow necrosis; Hyperhemolysis syndrome; Sickle cell disease