title: Red blood cell transfusions for thalassemia: results of a survey assessing current practice and proposal of evidence-based guidelines.
abstract: BACKGROUND
  In the absence of curative treatment, such as stem cell transplant, regular transfusions remain the mainstay of therapy for individuals with thalassemia major, a syndrome that results from marked ineffective erythropoiesis and the resultant anemia. The primary objectives of transfusion therapy are twofold: to suppress ineffective erythropoiesis and to ensure appropriate growth and development through childhood. In practice, a number of different transfusion protocols are in use across the developed world, with on-demand transfusion still being the paradigm in most of the developing world with limited resources.
  
  
  METHODS
  To investigate perceived differences in transfusion practice, a self-reported electronic survey was disseminated to eight US thalassemia treatment centers in February 2011. The survey was divided into sections ranging from laboratory and clinical practices to emerging transfusion-transmitted diseases.
  
  
  RESULTS
  The survey response rate was 100%. The total number of transfused patients was 411. One-hundred percent of institutions used leukoreduced blood. No centers routinely provided cytomegalovirus-seronegative red blood cells (RBCs). Half the centers provided irradiated RBCs; only one routinely provided washed RBCs, and none transfused RBCs of defined storage age. Seventy-five percent of centers routinely phenotyped thalassemia patients' RBC antigens; 50% prophylactically matched for Rh and K antigens. The frequency of antibody investigations varied widely, and 25% of centers routinely medicated patients before transfusion.
  
  
  CONCLUSIONS
  Eight thalassemia centers in the United States were surveyed to determine the uniformity of transfusion practice. The variability of the results was surprising. Consequently, we performed a literature review and propose an evidence-based protocol for routine transfusion therapy for patients with thalassemia.
authors.affiliation: Departments of Transfusion Medicine and Pediatrics, New York Presbyterian Hospital-Weill Cornell Medical College, New York, New York.
authors.forename: Cheryl
authors.initials: C
authors.lastname: Goss
authors.forename: Patricia
authors.initials: P
authors.lastname: Giardina
authors.forename: Diana
authors.initials: D
authors.lastname: Degtyaryova
authors.forename: Dorothy
authors.initials: D
authors.lastname: Kleinert
authors.forename: Sujit
authors.initials: S
authors.lastname: Sheth
authors.forename: Melissa
authors.initials: M
authors.lastname: Cushing
mesh_terms: D001793:Blood Preservation / Q000379:methods / Q000592:standards; D058731:Blood Safety / Q000379:methods / Q000592:standards; D003625:Data Collection; D017707:Erythrocyte Transfusion / Q000379:methods / Q000592:standards / Q000706:statistics & numerical data; D055317:Evidence-Based Practice / Q000592:standards; D006801:Humans; D017410:Practice Guidelines as Topic; D011364:Professional Practice / Q000592:standards / Q000706:statistics & numerical data; D011795:Surveys and Questionnaires; D013789:Thalassemia / Q000453:epidemiology / Q000628:therapy*; D014481:United States / Q000453:epidemiology