title: The role of molecular typing and perfect match transfusion in sickle cell disease and thalassaemia: An innovative transfusion strategy.
abstract: Chronic red blood cell transfusions remain an essential part of supportive treatment in patients with thalassaemia and sickle cell disease (SCD). Red blood cell (RBC) transfusions expose patients to the risk of developing antibodies: RBC alloimmunization occurs when the immune system meets foreign antigens. We created a register of extensively genotyped donors to achieve a better matched transfusion in order to reduce transfusion alloimmunization. Extended RBC antigen typing was determined and confirmed by molecular biology techniques using Human Erythrocyte Antigen (HEA) BeadChip (BioArray Solutions Ltd., Warren, NJ) in periodic blood donors and in patients with thalassaemia and SCD. During 3 years, we typed extensively 1220 periodic blood donors, 898 male and 322 female. We also studied 10 hematologic patients affected by thalassaemia and sickle cell disease referred to our institution as candidate to periodic transfusions. Our patients (8 females and 2 males with a median age of 48 years, range 24-76 years), extensively typed using molecular techniques and screened for RBC alloantibodies, were transfused with a median of 33.5 RBC units. After three years of molecular typing, the "perfect match" transfusion strategy avoided new alloantibodies development in all studied patients.
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy. Electronic address: rossana.putzulu@policlinicogemelli.it.
authors.forename: Rossana
authors.initials: R
authors.lastname: Putzulu
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Nicola
authors.initials: N
authors.lastname: Piccirillo
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Nicoletta
authors.initials: N
authors.lastname: Orlando
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Giuseppina
authors.initials: G
authors.lastname: Massini
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Maddalena
authors.initials: M
authors.lastname: Maresca
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Fernando
authors.initials: F
authors.lastname: Scavone
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Bianca Maria
authors.initials: BM
authors.lastname: Ricerca
authors.affiliation: Transfusion Medicine Department, Haematology Institute, Università Cattolica Sacro Cuore, Rome, Italy.
authors.forename: Gina
authors.initials: G
authors.lastname: Zini
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000755:Anemia, Sickle Cell / Q000235:genetics / Q000628:therapy; D017707:Erythrocyte Transfusion; D005260:Female; D060005:Genotyping Techniques / Q000379:methods*; D006801:Humans; D008297:Male; D008875:Middle Aged; D013789:Thalassemia / Q000235:genetics / Q000628:therapy; D065227:Transfusion Reaction / Q000235:genetics / Q000517:prevention & control*
keywords: Alloimmunization; Genotyping; RBC antigens and antibodies; Transfusion strategy