Original article

Vol. 22 No. 2 (2024): Blood Transfusion 2-2024 (March-April)

Transfusion in hemoglobinopathies and red blood cell alloimmunization, data from Sicily, Sardinia and Malta


Key words: haemoglobinopathy, thalassemia, sickle cell disease, alloimmunization, red cells transfusion, immunohematology
Publication Date: 2023-05-22


Background - Hemoglobinopathies are a group of diseases that include those due to globin gene mutations, such as thalassemia major (TM) and thalassemia intermedia (TI) or due to alteration of hemoglobin structure such as sickle cell disease (SCD), as well as a combination of these conditions such as thalasso-drepanocytosis (TD). They constitute the most frequent hereditary anemias requiring blood transfusion.
Materials and methods - In April 2022, a questionnaire was sent to the Transfusion Services (TS) of Sicily, Sardinia and the Maltese National Blood Transfusion (MNBT) service. The questionnaire was divided into a generic part including the number of patients followed and the type of hemoglobinopathy, and a section relating to transfusion therapy, including the number of units transfused, whether red blood cells (RBC) were washed and, finally, a section relating to the presence or absence of alloantibodies and their identification.
Results - Data was retrieved for 2,574 patients: 68.6% TM, 15.4% TI, 10.3% TD, 4.1% SCD, and 1.6% other hemoglobinopathies (OHA). The number of RBC units transfused was 76,974, equivalent to 24.5% of all the RBCU transfused from the total number of patients followed. The number of washed RBCU was 21.1% of all the units used; 337 patients (37%) were diagnosed with alloantibodies, the majority of which were patients with SCD (20.6%). Of the 485 alloantibodies found, 90.3% were identified. The antibodies found most frequently were related to the Kell system (41.7%) followed by antibodies to the Rhesus system (37.9%); 29.7% of patients had more than one antibody.
Discussion - From our study, certain indications can be formulated:
1) complete the National Registry for patients with hemoglobinopathies;
2) create a Registry of alloimmunized patients to ensure transfusion therapy is as safe as possible, considering antibody evanescence; and 3) increase the recruitment of blood donors of diverse ethnicities.



Giovanni Garozzo - Italian Society of Transfusion Medicine and Immunohematology (SIMTI), Regional Section of Sicily

Renato Messina - Italian Society of Transfusion Medicine and Immunohematology (SIMTI), Regional Section of Sicily

Pietro Carmelo Manca - Italian Society of Transfusion Medicine and Immunohematology (SIMTI), Regional Section of Sardinia

Alex Aquilina - Maltese National Blood Transfusion Service, St. Luke's Hospital, Guardamangia, Malta

Salvatore Platania - SIMT ASP 3, Caltagirone, Paternò, Italy

Filippo Buscemi - SIMT ASP 1, Agrigento, Italy

Gaetano Amodeo - SIMT ASP 1, Agrigento, Italy

Roberto Lisi - UOD Talassemia, ARNAS Garibaldi, Catania, Italy

Sebastiano Costanzo - SIMT AOU Policlinico “G. Rodolico-San Marco”, Catania, Italy

Noemi Agosto - SIMT AOR Villa Sofia-V. Cervello, Palermo, Italy

Nunzio Marletta - SIMT ASP 2, Caltanissetta, Gela, Italy

Francesco Bennardello - SIMT ASP 7, Ragusa, Italy

Vincenzo Spadola - UOD Talassemia, ASP 7, Ragusa, Italy

Graziella Vaccaro - SIMT ASP 9, Marsala, Italy

Teresa Barone - SIMT ASP 6, Cefalù, Italy

Francesco Spedale - SIMT ASP 4, Enna, Piazza Armerina, Italy

Vincenzo Barbera - SIMT ASP 4, Enna, Piazza Armerina, Italy

Eugenia Quartarone - SIMT AOU “G. Martino”, Messina, Italy

Annamaria Petrungaro - SIMT AOU “G. Martino”, Messina, Italy

Paolo Sciarrone - SIMT AOU “G. Martino”, Messina, Italy

Dario Genovese - SIMT ASP 8, Siracusa-Lentini, Italy

Antonio Ferrante - SIMT ARNAS Civico-Di Cristina-Benfratelli, Palermo, Italy

Gaetano Crisà - SIMT ASP 5, Patti, Italy

Maria Concetta Santoro - SIMT ASP 5, Taormina, Italy

Pasquale Gallerano - SIMT ASP 1, Sciacca, Italy

Sergio Bartoletti - SIMT ASSL Sassari-Ozieri, Italy

Angelo Zuccarelli - SIMT ASSL Sulcis, Italy

Angela Marras - SIMT AO Brotzu, Cagliari, Italy

Isabella Atzeni - SIMT ASSL Sanluri, Italy

Marco Cocco - SIMT ASSL Olbia, Italy

Mauro Murgia - SIMT ASSL Oristano, Italy

Pierpaolo Bitti - SIMT ASSL Nuoro, Italy

Monique Debattista - National Blood Center, Malta

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