title: Does a patient with hereditary spherocytosis qualify for preoperative autologous blood donation?
abstract: BACKGROUND
  Hereditary spherocytosis (HS) is characterized by osmotically fragile spherocytic red cells (RBCs), mild to moderate hemolysis, and splenomegaly. Little is known about the safety of blood bank storage of HS RBCs.
  
  
  METHODS
  A 50-year-old man with mild autosomally dominant HS asked to make an autologous RBC deposit before his scheduled surgery. His RBCs were serially tested for osmotic fragility during 4 weeks of blood bank storage in CPD with adenine-saline. Aliquots of his fresh and 4-week-old RBCs were also labeled with 51Cr for measurement of in vivo survival. The osmotic fragility of the patient's fresh RBCs was normal, with 50-percent hemolysis at approximately 0.43-percent NaCl and a sigmoid lysis curve. Incubated osmotic fragility (24 hours at 37 degrees C) was abnormal, with 50-percent hemolysis at 0.64-percent NaCl. Median survival of his fresh 51Cr-labeled RBCs was moderately shortened at 17.5 days. Osmotic fragility increased during storage, with 50-percent lysis occurring at 0.58-percent and 0.62-percent NaCl after 2 and 4 weeks, respectively. Volunteer normal donor RBCs exhibited 50-percent lysis in 0.48-percent NaCl after 4 weeks. Median survival of the patient's 4-week-old 51Cr-labeled RBCs was severely shortened, at 3.5 days. At surgery, intraoperatively salvaged RBCs demonstrated osmotic fragility identical to that of the patient's RBCs freshly obtained by venipuncture.
  
  
  CONCLUSIONS
  HS RBCs may lose membrane under optimum storage conditions, becoming unsuitable for transfusion at surgery. Intraoperative autologous transfusion may be preferable to preoperative deposit for avoidance of allogeneic RBC transfusion in patients with HS.
authors.affiliation: Department of Medicine, St. Elizabeth's Medical Center of Boston, Tufts University School of Medicine, Massachusetts, USA.
authors.forename: R
authors.initials: R
authors.lastname: Weinstein
authors.forename: R
authors.initials: R
authors.lastname: Martinez
authors.forename: H
authors.initials: H
authors.lastname: Hassoun
authors.forename: J
authors.initials: J
authors.lastname: Palek
mesh_terms: D001457:Anion Exchange Protein 1, Erythrocyte / Q000032:analysis; D001782:Blood Donors; D001793:Blood Preservation / Q000009:adverse effects; D001797:Blood Protein Electrophoresis; D001804:Blood Transfusion, Autologous / Q000592:standards*; D004905:Erythrocyte Aging / Q000502:physiology; D006461:Hemolysis / Q000502:physiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009996:Osmotic Fragility / Q000502:physiology; D011300:Preoperative Care; D013049:Spectrin / Q000032:analysis; D013103:Spherocytosis, Hereditary / Q000097:blood* / Q000235:genetics
chemical_list: D001457:Anion Exchange Protein 1, Erythrocyte; D013049:Spectrin