title: Successful treatment of recurrent hyperhemolysis syndrome with immunosuppression and plasma-to-red blood cell exchange transfusion.
abstract: BACKGROUND
  Hyperhemolysis syndrome is a serious transfusion reaction mostly reported in association with sickle cell disease, characterized by destruction of both donor and host red blood cells (RBCs) by an unknown mechanism.
  
  
  METHODS
  A 21-year-old man with sickle cell disease and multiple prior transfusions received two phenotype-matched, compatible RBC units during a brief admission for pain crisis. He developed rapid-onset progressive anemia and hemoglobinuria. Methylprednisolone, erythropoietin, and rituximab were administered. Fifteen days posttransfusion the hemoglobin (Hb) concentration decreased to 3.1 g/dL, with evidence of severe congestive heart failure. No new antibodies were identified. It was felt that his heart failure would not improve without increasing oxygen-carrying capacity. A combination of volume overload, anemia, and hemolysis prompted a novel isovolemic procedure to increase Hb level without removing his own RBCs or causing fluid overload. A cell separator was used operating on the plasma-exchange program, with three cross-match-compatible, washed RBC units as the replacement fluid. After the procedure, there was no evidence of hemolysis. Over the following 6 days, the congestive heart failure resolved, the Hb concentration increased to 7.5 g/dL, and the patient fully recovered. He had a similar event 3 years previously.
  
  
  CONCLUSIONS
  Plasma-to-RBC replacement may be beneficial for selected patients with life-threatening anemia. This intervention provides immediate improvement in oxygen-carrying capacity, conserving the patient's own RBCs, while avoiding fluid overload. Although blood transfusion may precipitate further hemolysis, this case report describes successful plasma-to-RBC exchange transfusion with concurrent supportive care to offset hemolysis, including corticosteroid, intravenous immunoglobulin, and rituximab.
authors.affiliation: Transfusion Medicine and Pheresis Unit, Beth Israel Deaconess Medical Center, Boston, Massachusetts; Departments of Hematology, Oncology and Bone Marrow Transplant, St Louis Children's Hospital, St Louis, Missouri; Departments of Pathology and Medicine, Stanford University Medical Center, Stanford, California.
authors.forename: Erik J
authors.initials: EJ
authors.lastname: Uhlmann
authors.forename: Shalini
authors.initials: S
authors.lastname: Shenoy
authors.forename: Lawrence T
authors.initials: LT
authors.lastname: Goodnough
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000755:Anemia, Sickle Cell / Q000276:immunology* / Q000628:therapy*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001787:Blood Group Incompatibility / Q000188:drug therapy* / Q000276:immunology; D017707:Erythrocyte Transfusion / Q000009:adverse effects*; D006461:Hemolysis / Q000187:drug effects / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D010951:Plasma Exchange / Q000379:methods*; D012008:Recurrence; D000069283:Rituximab; D013577:Syndrome; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D000069283:Rituximab