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1. Luspatercept for the treatment of anaemia in non-transfusion-dependent β-thalassaemia (BEYOND): a phase 2, randomised, double-blind, multicentre, placebo-controlled trial

Author

Buaboonnam, Jassada, Ekwattanakit, Supachai, Khunhapinant, Archrob, Loka, Efthalia, Moraki, Maria, Flevari, Pagona, Dimopoulou, Maria, Bartzi, Vasiliki, Daadaa, Hisham, El Hasbani, Georges, Koussa, Suzanne, Tartaglione, Immacolata, Ammendola, Federica, Scianguetta, Saverio, Puglia, Marta, Ferrara, Ilaria, Ferrero, Giovanni, Gaglioti, Carmen, Longo, Filomena, Turrini, Silvia, Voi, Vincenzo, Cassinerio, Elena, De, Anna, Graziadei, Giovanna, Marcon, Alessia, Migone De Amicis, Margherita, Motta, Irene, Cinque, Patrizia, Pannone, Bruno, Ricchi, Paolo, Balocco, Manuela, Carrara, Paola, Della Rovere, Francesco, Lamagna, Martina, Pinto, Valeria, Quintino, Sabrina, Eleftheriou, Perla, Garbowski, Maciej, de Kreuk, Arne, Carson, Susan, Denton, Christopher, Hofstra, Tom, Veluswamy, Sayany, Wood, John, Badawy, Sherif, Bercovitz, Rachel, Bhat, Rukhmi, Calamaras, Diane, Liem, Robert, Mack, Astrid, Taher, Ali T, Cappellini, Maria Domenica, Kattamis, Antonis, Voskaridou, Ersi, Perrotta, Silverio, Piga, Antonio G, Filosa, Aldo, Porter, John B, Coates, Thomas D, Forni, Gian Luca, Thompson, Alexis A, Musallam, Khaled M, Backstrom, Jay T, Esposito, Oriana, Giuseppi, Ana Carolina, Kuo, Wen-Ling, Miteva, Dimana, Lord-Bessen, Jennifer, Yucel, Aylin, Zinger, Tatiana, Shetty, Jeevan K, and Viprakasit, Vip

Published
2022
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3. Hydroxyurea Pharmacokinetic Evaluation in Patients with Sickle Cell Disease.

Author

Di Grazia, Daniela, Mirabella, Cristina, Chiara, Francesco, Caudana, Maura, Shelton Agar, Francesco Maximillian Anthony, Zanatta, Marina, Allegra, Sarah, Bertello, Jenni, Voi, Vincenzo, Ferrero, Giovanni Battista, Abbadessa, Giuliana, and De Francia, Silvia

Subjects
SICKLE cell anemia, DRUG monitoring, BODY surface area, BODY mass index, AGE differences
Abstract

Background: Hydroxyurea (HU), also known as hydroxycarbamide, is an oral ribonucleotide reductase inhibitor. In 1999, the United States Food and Drug Administration (FDA) approved HU for the treatment of sickle cell disease (SCD). Since then, it has become the cornerstone in the management of SCD patients, helping to reduce vaso-occlusive crises, acute chest syndrome, the need for blood transfusions, hospitalizations and mortality. There is considerable variability among individuals in HU pharmacokinetic (Pk) parameters that can influence treatment efficacy and toxicity. The objective of this work is part of a clinical study aimed at investigating HU Pk and determining the optimal sampling time to estimate the Area Under the Curve (AUC) in SCD patients. Methods: HU plasma concentration in 80 patients at various time points (2, 4, 6, 24 h) following a 48-h drug washout was quantified using High-Pressure Liquid Chromatography (HPLC) coupled with an ultraviolet (UV) detection method previously described in the literature and adapted to new conditions with partial modifications. Results: The mean HU administered dose was 19.5 ± 5.1 mg/kg (range: 7.7–37.5 mg/kg). The median AUC quantified in plasma patients was 101.3 mg/L/h (Interquartile Range (IQR): 72.5–355.9) and it was not influenced by the weight-based dose. However, there was a strong positive correlation between AUC and Body Mass Index (BMI) as well as dose per Body Surface Area (BSA). Along with a three-point approach for AUC determination present in the literature, we show results obtained from a four-point sampling strategy, which is more useful and effective for better optimizing dose escalation to the maximum tolerated dose (MTD). Moreover, we observed that most patients achieved the maximum HU plasma concentration two hours after drug administration, regardless of age differences. Conclusions: HU treatment, which represents a milestone in the treatment of SCD due to its ability to reduce disease complications and improve patients' quality of life, requires careful monitoring to optimize the individual dose for saving potential side effects and/or adverse events. [ABSTRACT FROM AUTHOR]

Published
2024
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6. Current challenges in the management of patients with sickle cell disease – A report of the Italian experience

Author

Russo, Giovanna, De Franceschi, Lucia, Colombatti, Raffaella, Rigano, Paolo, Perrotta, Silverio, Voi, Vincenzo, Palazzi, Giovanni, Fidone, Carmelo, Quota, Alessandra, Graziadei, Giovanna, Pietrangelo, Antonello, Pinto, Valeria, Ruffo, Giovan Battista, Sorrentino, Francesco, Venturelli, Donatella, Casale, Maddalena, Ferrara, Francesca, Sainati, Laura, Cappellini, Maria Domenica, Piga, Antonio, Maggio, Aurelio, and Forni, Gian Luca

Published
2019
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7. Nephrological Complications in Hemoglobinopathies: SITE Good Practice.

Author

Ruffo, Giovan Battista, Russo, Rodolfo, Casini, Tommaso, Lombardini, Letizia, Orecchia, Valeria, Voi, Vincenzo, Origa, Raffaella, Forni, Gian Luca, Marchetti, Monia, Gigante, Antonia, Garibotto, Giacomo, Maggio, Aurelio, and De Franceschi, Lucia

Subjects
KIDNEY diseases, CHRONIC kidney failure, SICKLE cell anemia, LITERATURE reviews, KIDNEY transplantation, COLOR codes, INTERNAL migration
Abstract

Background. Hemoglobinopathies, among which thalassemic syndromes (transfusion-dependent and non-transfusion dependent thalassemias) and sickle cell disease (SCD), are the most widespread monogenic diseases worldwide. Hemoglobinopathies are endemic and spread-out all-over Italy, as result of internal and external migration flows. Nowadays, the increase therapeutic options associated to the general aging of patients with hemoglobinopathies related to the improvement in clinical management, contribute to the abnormalities in kidney function going from blood and urine test alterations to chronic kidney disease and end stage renal disease. Methods. Here, we carried out a revision of the literature as panel of recognized experts in hemoglobinopathies with the consultancy and the revision of two nephrologists on kidney alteration and kidney disease in patients with TDT, NTDT and SCD. This is part of the action of the Italian society for the study of thalassemia and hemoglobinopties (SITE). The purpose of this "good practice (GP)" is to provide recommendations for follow-up and therapy for the management of kidney alterations in patients with TDT, NTDT and SCD. The literature review covers the period 1.1.2016 to 31.12.2022. In consideration of the rarity of these diseases, the analysis was extended from 5 to 7 years. Moreover, in the absence of relevant scientific papers in the identified time frame, we referred to pivotal or population studies, when available. Finally, in the absence of evidence-based data from prospective and randomized trials, the authors had to refer to expert opinion (expert consensus) for many topics. Results. We generated question and answer boxes to offer a friendly consultation, using color code strategy and focused answers. Conclusions. The present GP will help in improving the clinical management, and the quality of care of patients with hemoglobinopathies. [ABSTRACT FROM AUTHOR]

Published
2023
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9. Luspatercept for the treatment of anaemia in non-transfusion-dependent β-thalassaemia (BEYOND): a phase 2, randomised, double-blind, multicentre, placebo-controlled trial

Author

Taher, Ali T, primary, Cappellini, Maria Domenica, additional, Kattamis, Antonis, additional, Voskaridou, Ersi, additional, Perrotta, Silverio, additional, Piga, Antonio G, additional, Filosa, Aldo, additional, Porter, John B, additional, Coates, Thomas D, additional, Forni, Gian Luca, additional, Thompson, Alexis A, additional, Tartaglione, Immacolata, additional, Musallam, Khaled M, additional, Backstrom, Jay T, additional, Esposito, Oriana, additional, Giuseppi, Ana Carolina, additional, Kuo, Wen-Ling, additional, Miteva, Dimana, additional, Lord-Bessen, Jennifer, additional, Yucel, Aylin, additional, Zinger, Tatiana, additional, Shetty, Jeevan K, additional, Viprakasit, Vip, additional, Buaboonnam, Jassada, additional, Ekwattanakit, Supachai, additional, Khunhapinant, Archrob, additional, Loka, Efthalia, additional, Moraki, Maria, additional, Flevari, Pagona, additional, Dimopoulou, Maria, additional, Bartzi, Vasiliki, additional, Daadaa, Hisham, additional, El Hasbani, Georges, additional, Koussa, Suzanne, additional, Ammendola, Federica, additional, Scianguetta, Saverio, additional, Puglia, Marta, additional, Ferrara, Ilaria, additional, Ferrero, Giovanni, additional, Gaglioti, Carmen, additional, Longo, Filomena, additional, Turrini, Silvia, additional, Voi, Vincenzo, additional, Cassinerio, Elena, additional, De, Anna, additional, Graziadei, Giovanna, additional, Marcon, Alessia, additional, Migone De Amicis, Margherita, additional, Motta, Irene, additional, Cinque, Patrizia, additional, Pannone, Bruno, additional, Ricchi, Paolo, additional, Balocco, Manuela, additional, Carrara, Paola, additional, Della Rovere, Francesco, additional, Lamagna, Martina, additional, Pinto, Valeria, additional, Quintino, Sabrina, additional, Eleftheriou, Perla, additional, Garbowski, Maciej, additional, de Kreuk, Arne, additional, Carson, Susan, additional, Denton, Christopher, additional, Hofstra, Tom, additional, Veluswamy, Sayany, additional, Wood, John, additional, Badawy, Sherif, additional, Bercovitz, Rachel, additional, Bhat, Rukhmi, additional, Calamaras, Diane, additional, Liem, Robert, additional, and Mack, Astrid, additional

Published
2022
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10. Thalassaemia is paradoxically associated with a reduced risk of in‐hospital complications and mortality in COVID‐19: Data from an international registry

Author

El‐Battrawy, Ibrahim, primary, Longo, Filomena, additional, Núñez Gil, Iván J., additional, Abumayyaleh, Mohammad, additional, Gianesin, Barbara, additional, Estrada, Vicente, additional, Aparisi, Álvaro, additional, Arroyo‐Espliguero, Ramón, additional, Balocco, Manuela, additional, Barella, Susanna, additional, Beccaria, Andrea, additional, Bonetti, Federico, additional, Casale, Maddalena, additional, De michele, Elisa, additional, Denotti, Anna Rita, additional, Fidone, Carmelo, additional, Fortini, Monica, additional, Gamberini, Maria Rita, additional, Graziadei, Giovanna, additional, Lisi, Roberto, additional, Massa, Antonella, additional, Marcon, Alessia, additional, Rubinski, Bryan, additional, Miano, Maurizio, additional, Motta, Irene, additional, Pinto, Valeria Maria, additional, Piperno, Alberto, additional, Mariani, Raffaella, additional, Putti, Maria Caterina, additional, Quota, Alessandra, additional, Ribersani, Michela, additional, Marziali, Marco, additional, Roberti, Domenico, additional, Rosso, Rosamaria, additional, Tartaglione, Immacolata, additional, Vitucci, Angelantonio, additional, Voi, Vincenzo, additional, Zecca, Marco, additional, Romero, Rodolfo, additional, Marouneld, Charbel, additional, Fernández‐Rozas, Inmaculada, additional, Espejo, Carolina, additional, Marhaeni, Wulandewi, additional, Garcia Aguado, Marcos, additional, Cappellini, Maria Domenica, additional, Perrotta, Silverio, additional, De Franceschi, Lucia, additional, Piga, Antonio, additional, Forni, Gian Luca, additional, and Akin, Ibrahim, additional

Published
2022
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11. Transfusional Approach in Multi-Ethnic Sickle Cell Patients: Real-World Practice Data From a Multicenter Survey in Italy

Author

Graziadei, Giovanna, primary, De Franceschi, Lucia, additional, Sainati, Laura, additional, Venturelli, Donatella, additional, Masera, Nicoletta, additional, Bonomo, Piero, additional, Vassanelli, Aurora, additional, Casale, Maddalena, additional, Lodi, Gianluca, additional, Voi, Vincenzo, additional, Rigano, Paolo, additional, Pinto, Valeria Maria, additional, Quota, Alessandra, additional, Notarangelo, Lucia D., additional, Russo, Giovanna, additional, Allò, Massimo, additional, Rosso, Rosamaria, additional, D'Ascola, Domenico, additional, Facchini, Elena, additional, Macchi, Silvia, additional, Arcioni, Francesco, additional, Bonetti, Federico, additional, Rossi, Enza, additional, Sau, Antonella, additional, Campisi, Saveria, additional, Colarusso, Gloria, additional, Giona, Fiorina, additional, Lisi, Roberto, additional, Giordano, Paola, additional, Boscarol, Gianluca, additional, Filosa, Aldo, additional, Marktel, Sarah, additional, Maroni, Paola, additional, Murgia, Mauro, additional, Origa, Raffaella, additional, Longo, Filomena, additional, Bortolotti, Marta, additional, Colombatti, Raffaella, additional, Di Maggio, Rosario, additional, Mariani, Raffaella, additional, Piperno, Alberto, additional, Corti, Paola, additional, Fidone, Carmelo, additional, Palazzi, Giovanni, additional, Badalamenti, Luca, additional, Gianesin, Barbara, additional, Piel, Frédéric B., additional, and Forni, Gian Luca, additional

Published
2022
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12. Occult ischemic bone lesions in children with sickle cell disease: A study of prevalence.

Author

Voi, Vincenzo, Turrini, Silvia, Mattavelli, Margherita, Vigliani, Valentina, Margarita, Giuseppe, and Ferrero, Giovanni Battista

Subjects
*SICKLE cell anemia, *IDIOPATHIC femoral necrosis, *DISEASE prevalence, *MAGNETIC resonance imaging, *FEMUR head, *ODDS ratio, *SKELETAL maturity
Abstract

Introduction: Avascular necrosis (AVN) is a severe complication of sickle cell disease (SCD) and involves principally the femoral head. Few data exist about the prevalence of lesions in other segments. Methods: In this cross‐sectional study, 42 children (20 males and 22 females) underwent a magnetic resonance imaging (MRI) of the spine, upper arms, and femurs. The primary outcome was to define the prevalence and locations of bone infarcts. Results: Forty‐two patients completed the study; the total median age was 11.9 years (interquartile range = 9.5–13.9). Eleven patients (26.2%) were positive for altered bone findings for a total of 32 lesions. Most of the lesions were in the humerus 17 (53.1%), 11 (34.4%) in the femurs, and 4 (12.5%) in the vertebrae. The median number of vaso‐occlusive crises (VOCs) was two and four for patients without and with bone lesions, respectively (p =.01). The annual rate of VOC is the best marker for the positive MRI (odds ratio = 82.6; p =.03), and it is correlated with the number of sites involved (p =.02). Conclusions: Our study highlights that the prevalence of skeletal lesions could be underestimated, and it provides the basis for clinical reasoning and tailored therapy in SCD children. [ABSTRACT FROM AUTHOR]

Published
2023
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13. Italian patients with hemoglobinopathies exhibit a 5‐fold increase in age‐standardized lethality due to SARS‐CoV‐2 infection

Author

Longo, Filomena, primary, Gianesin, Barbara, additional, Voi, Vincenzo, additional, Motta, Irene, additional, Pinto, Valeria Maria, additional, Piolatto, Andrea, additional, Spasiano, Anna, additional, Ruffo, Giovan Battista, additional, Gamberini, Maria Rita, additional, Barella, Susanna, additional, Mariani, Raffaella, additional, Fidone, Carmelo, additional, Rosso, Rosamaria, additional, Casale, Maddalena, additional, Roberti, Domenico, additional, Dal Zotto, Chiara, additional, Vitucci, Angelantonio, additional, Bonetti, Federico, additional, Pitrolo, Lorella, additional, Quaresima, Micol, additional, Ribersani, Michela, additional, Quota, Alessandra, additional, Arcioni, Francesco, additional, Campisi, Saveria, additional, Massa, Antonella, additional, De Michele, Elisa, additional, Lisi, Roberto, additional, Miano, Maurizio, additional, Bagnato, Sabrina, additional, Gentile, Massimo, additional, Carrai, Valentina, additional, Putti, Maria Caterina, additional, Serra, Marilena, additional, Gaglioti, Carmen, additional, Migone De Amicis, Margerita, additional, Graziadei, Giovanna, additional, De Giovanni, Anna, additional, Ricchi, Paolo, additional, Balocco, Manuela, additional, Quintino, Sabrina, additional, Borsellino, Zelia, additional, Fortini, Monica, additional, Denotti, Anna Rita, additional, Tartaglione, Immacolata, additional, Beccaria, Andrea, additional, Marziali, Marco, additional, Maggio, Aurelio, additional, Perrotta, Silverio, additional, Piperno, Alberto, additional, Filosa, Aldo, additional, Cappellini, Maria Domenica, additional, De Franceschi, Lucia, additional, Piga, Antonio, additional, and Forni, Gian Luca, additional

Published
2021
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14. Limited Access to Transcranial Doppler Screening and Stroke Prevention for Children with Sickle Cell Disease in Europe: Results of a Multinational Eurobloodnet Survey

Author

Cuzzubbo, Daniela, primary, Gutierrez-Valle, Victoria, additional, Casale, Maddalena, additional, Voi, Vincenzo, additional, McMahon, Corrina, additional, Mañú Pereira, Maria Del Mar, additional, de Montalembert, Mariane, additional, Inusa, Baba PD, additional, and Colombatti, Raffaella, additional

Published
2021
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15. Thalassemia Is Paradoxically Associated with a Reduced Risk of In-Hospital Complications and Mortality in COVID-19: Data from an International Registry

Author

El-Battrawy, Ibrahim, primary, Longo, Filomena, additional, Núñez Gil, Iván, additional, Abumayyaleh, Mohammad, additional, Gianesin, Barbara, additional, Estrada, Vicente, additional, Aparisi, Alvaro, additional, Arroyo-Espliguero, Ramón, additional, Balocco, Manuela, additional, Barella, Susanna, additional, Beccaria, Andrea, additional, Bonetti, Federico, additional, Casale, Maddalena, additional, De Michele, Elisa, additional, Denotti, Anna Rita, additional, Fidone, Carmelo, additional, Fortini, Monica, additional, Gamberini, Rita, additional, Graziadei, Giovanna, additional, Lisi, Roberto, additional, Massa, Antonella, additional, Marcon, Alessia, additional, Rupinski, Bryan, additional, Miano, Maurizio, additional, Motta, Irene, additional, Pinto, Valeria Maria, additional, Piperno, Alberto, additional, Mariani, Raffaella, additional, Putti, Maria Caterina, additional, Quota, Alessandra, additional, Ribersani, Michela, additional, Marziali, Marco, additional, Roberti, Domenico, additional, Rosso, Rosamaria, additional, Tartaglione, Immacolata, additional, Vitucci, Angelantonio, additional, Voi, Vincenzo, additional, Zecca, Marco, additional, Romero, Rodolfo, additional, Maroun, Charbel, additional, Fernández-Rozas, Inmaculada, additional, Paeres, Carolina Espejo, additional, Marhaeni, Wulandewi, additional, Garca-Aguado, Marcos, additional, Cappellini, Maria Domenica, additional, Perrotta, Silverio, additional, De Franceschi, Lucia, additional, Piga, Antonio, additional, Forni, Gian Luca, additional, and Akin, Ibrahim, additional

Published
2021
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16. Italian patients with hemoglobinopathies exhibit a 5‐fold increase in age‐standardized lethality due to SARS‐CoV‐2 infection.

Author

Longo, Filomena, Gianesin, Barbara, Voi, Vincenzo, Motta, Irene, Pinto, Valeria Maria, Piolatto, Andrea, Spasiano, Anna, Ruffo, Giovan Battista, Gamberini, Maria Rita, Barella, Susanna, Mariani, Raffaella, Fidone, Carmelo, Rosso, Rosamaria, Casale, Maddalena, Roberti, Domenico, Dal Zotto, Chiara, Vitucci, Angelantonio, Bonetti, Federico, Pitrolo, Lorella, and Quaresima, Micol

Published
2022
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17. Acute events in children with sickle cell disease in Italy during the COVID‐19 pandemic: useful lessons learned.

Author

Munaretto, Vania, Voi, Vincenzo, Palazzi, Giovanni, Notarangelo, Lucia Dora, Corti, Paola, Baretta, Valentina, Casale, Maddalena, Barone, Angelica, Cuzzubbo, Daniela, Samperi, Piera, Tripodi, Serena, Giona, Fiorina, Miano, Maurizio, Nocerino, Agostino, Del Vecchio, Gian Carlo, Piccolo, Chiara, Sau, Antonella, Filippini, Beatrice, Casciana, Maria Luisa, and Arcioni, Francesco

Subjects
*COVID-19 pandemic, *SICKLE cell anemia, *COVID-19, *SYMPTOMS, *INTRA-abdominal hypertension, *INFECTION prevention
Abstract

Acute events in children with sickle cell disease in Italy during the COVID-19 pandemic: useful lessons learned Acute chest, children, COVID-19, Italy, sickle cell disease, Vaso-occlusive crisis Keywords: acute chest; children; COVID-19; Italy; sickle cell disease; Vaso-occlusive crisis EN acute chest children COVID-19 Italy sickle cell disease Vaso-occlusive crisis 851 854 4 09/02/21 20210901 NES 210901 On March 12th 2021 the Italian Government decided to implement a national lockdown in almost all the regions of the country. [Extracted from the article]

Published
2021
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20. Transfusion Therapy in a Multi-Ethnic Sickle Cell Population Real-World Practice. a Preliminary Data Analysis of Multicentre Survey

Author

Graziadei, Giovanna, primary, Sainati, Laura, additional, Bonomo, Pietro, additional, Venturelli, Donatella, additional, Masera, Nicoletta, additional, Casale, Maddalena, additional, Vassanelli, Aurora, additional, Lodi, Gianluca, additional, Piel, Frédéric B, additional, Voi, Vincenzo, additional, De Franceschi, Lucia, additional, Rigano, Paolo, additional, Quota, Alessandra, additional, Notarangelo, Lucia Dora, additional, Russo, Giovanna, additional, Rosso, Rosamaria, additional, Allò, Massimo, additional, D'Ascola, Domenico, additional, Facchini, Elena, additional, Macchi, Silvia, additional, Arcioni, Francesco, additional, Piperno, Alberto, additional, Bonetti, Federico, additional, Palazzi, Giovanni, additional, Bisconte, Maria Grazia, additional, Sau, Antonella, additional, Lisi, Roberto, additional, Giona, Fiorina, additional, Campisi, Saveria, additional, Colarusso, Gloria, additional, Giordano, Paola, additional, Boscariol, Gianluca, additional, Marktel, Sarah, additional, Filosa, Aldo, additional, Origa, Raffaella, additional, Murgia, Mauro, additional, Maroni, Paola, additional, Gianesin, Barbara, additional, Badalamenti, Luca, additional, and Forni, Gian Luca, additional

Published
2018
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22. Transfusional Approach in Multi-Ethnic Sickle Cell Patients: Real-World Practice Data From a Multicenter Survey in Italy

Author

Giovanna Graziadei, Lucia De Franceschi, Laura Sainati, Donatella Venturelli, Nicoletta Masera, Piero Bonomo, Aurora Vassanelli, Maddalena Casale, Gianluca Lodi, Vincenzo Voi, Paolo Rigano, Valeria Maria Pinto, Alessandra Quota, Lucia D. Notarangelo, Giovanna Russo, Massimo Allò, Rosamaria Rosso, Domenico D'Ascola, Elena Facchini, Silvia Macchi, Francesco Arcioni, Federico Bonetti, Enza Rossi, Antonella Sau, Saveria Campisi, Gloria Colarusso, Fiorina Giona, Roberto Lisi, Paola Giordano, Gianluca Boscarol, Aldo Filosa, Sarah Marktel, Paola Maroni, Mauro Murgia, Raffaella Origa, Filomena Longo, Marta Bortolotti, Raffaella Colombatti, Rosario Di Maggio, Raffaella Mariani, Alberto Piperno, Paola Corti, Carmelo Fidone, Giovanni Palazzi, Luca Badalamenti, Barbara Gianesin, Frédéric B. Piel, Gian Luca Forni, Graziadei, Giovanna, De Franceschi, Lucia, Sainati, Laura, Venturelli, Donatella, Masera, Nicoletta, Bonomo, Piero, Vassanelli, Aurora, Casale, Maddalena, Lodi, Gianluca, Voi, Vincenzo, Rigano, Paolo, Pinto, Valeria Maria, Quota, Alessandra, Notarangelo, Lucia D, Russo, Giovanna, Allò, Massimo, Rosso, Rosamaria, D'Ascola, Domenico, Facchini, Elena, Macchi, Silvia, Arcioni, Francesco, Bonetti, Federico, Rossi, Enza, Sau, Antonella, Campisi, Saveria, Colarusso, Gloria, Giona, Fiorina, Lisi, Roberto, Giordano, Paola, Boscarol, Gianluca, Filosa, Aldo, Marktel, Sarah, Maroni, Paola, Murgia, Mauro, Origa, Raffaella, Longo, Filomena, Bortolotti, Marta, Colombatti, Raffaella, Di Maggio, Rosario, Mariani, Raffaella, Piperno, Alberto, Corti, Paola, Fidone, Carmelo, Palazzi, Giovanni, Badalamenti, Luca, Gianesin, Barbara, Piel, Frédéric B, and Forni, Gian Luca

Subjects
multi-ethnicity, transfusion therapy, alloimmunization, hydroxycarbamide, sickle cell disease, General Medicine
Abstract

Sickle cell disease (SCD) is a worldwide distributed hereditary red cell disorder characterized by recurrent acute vaso-occlusive crises (VOCs and anemia). Gold standard treatments are hydroxycarbamide (HC) and/or different red blood cell (RBC) transfusion regimens to limit disease progression. Here, we report a retrospective study on 1,579 SCD patients (median age 23 years; 802 males/777 females), referring to 34 comprehensive Italian centers for hemoglobinopathies. Although we observed a similar proportion of Caucasian (47.9%) and African (48.7%) patients, Italian SCD patients clustered into two distinct overall groups: children of African descent and adults of Caucasian descent. We found a subset of SCD patients requiring more intensive therapy with a combination of HC plus chronic transfusion regimen, due to partial failure of HC treatment alone in preventing or reducing sickle cell-related acute manifestations. Notably, we observed a higher use of acute transfusion approaches for SCD patients of African descent when compared to Caucasian subjects. This might be related to (i) age of starting HC treatment; (ii) patients' low social status; (iii) patients' limited access to family practitioners; or (iv) discrimination. In our cohort, alloimmunization was documented in 135 patients (8.5%) and was more common in Caucasians (10.3%) than in Africans (6.6%). Alloimmunization was similar in male and female and more frequent in adults than in children. Our study reinforces the importance of donor-recipient exact matching for ABO, Rhesus, and Kell antigen systems for RBC compatibility as a winning strategy to avoid or limit alloimmunization events that negatively impact the clinical management of SCD-related severe complications.Clinical Trial RegistrationClinicalTrials.gov, identifier: NCT03397017.

Published
2022
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23. Italian patients with hemoglobinopathies exhibit a 5-fold increase in age-standardized lethality due to SARS-CoV-2 infection

Author

Sabrina Quintino, Filomena Longo, Maurizio Miano, Vincenzo Voi, Maria Caterina Putti, Margerita Migone De Amicis, Monica Fortini, Susanna Barella, Barbara Gianesin, Federico Bonetti, Andrea Beccaria, Manuela Balocco, Andrea Piolatto, Saveria Campisi, Rosamaria Rosso, Angelantonio Vitucci, Valentina Carrai, Alessandra Quota, Zelia Borsellino, Antonio Piga, Maddalena Casale, Anna Rita Denotti, Michela Ribersani, Maria Rita Gamberini, Domenico Roberti, Alberto Piperno, Roberto Lisi, Carmelo Fidone, Maria Domenica Cappellini, Sabrina Bagnato, Anna De Giovanni, Micol Quaresima, Valeria Maria, Lorella Pitrolo, Marco Marziali, Giovanna Graziadei, Carmen Gaglioti, Aldo Filosa, Chiara Dal Zotto, Lucia De Franceschi, Irene Motta, Immacolata Tartaglione, Francesco Arcioni, Aurelio Maggio, Marilena Serra, Giovan Battista Ruffo, Massimo Gentile, Elisa De Michele, Anna Spasiano, Paolo Ricchi, Antonella Massa, Silverio Perrotta, R. Mariani, Gian Luca Forni, Longo, Filomena, Gianesin, Barbara, Voi, Vincenzo, Motta, Irene, Pinto, Valeria Maria, Piolatto, Andrea, Spasiano, Anna, Ruffo, Giovan Battista, Gamberini, Maria Rita, Barella, Susanna, Mariani, Raffaella, Fidone, Carmelo, Rosso, Rosamaria, Casale, Maddalena, Roberti, Domenico, Dal Zotto, Chiara, Vitucci, Angelantonio, Bonetti, Federico, Pitrolo, Lorella, Quaresima, Micol, Ribersani, Michela, Quota, Alessandra, Arcioni, Francesco, Campisi, Saveria, Massa, Antonella, De Michele, Elisa, Lisi, Roberto, Miano, Maurizio, Bagnato, Sabrina, Gentile, Massimo, Carrai, Valentina, Putti, Maria Caterina, Serra, Marilena, Gaglioti, Carmen, Migone De Amicis, Margerita, Graziadei, Giovanna, De Giovanni, Anna, Ricchi, Paolo, Balocco, Manuela, Quintino, Sabrina, Borsellino, Zelia, Fortini, Monica, Denotti, Anna Rita, Tartaglione, Immacolata, Beccaria, Andrea, Marziali, Marco, Maggio, Aurelio, Perrotta, Silverio, Piperno, Alberto, Filosa, Aldo, Cappellini, Maria Domenica, De Franceschi, Lucia, Piga, Antonio, and Forni, Gian Luca

Subjects
Sars-CoV-2, business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Sars-CoV-2, hemoglobinopathies, Medicine, hemoglobinopathies,SARS-CoV-2 infection, Lethality, Hematology, hemoglobinopathies, business, Virology
Published
2022

24. Thalassemia Is Paradoxically Associated with a Reduced Risk of In-Hospital Complications and Mortality in COVID-19: Data from an International Registry

Author

Ibrahim El-Battrawy, Elisa De Michele, Manuela Balocco, Immacolata Tartaglione, Carmelo Fidone, Roberto Lisi, Maria Caterina Putti, Marcos Garca-Aguado, Michela Ribersani, Ramón Arroyo-Espliguero, Vicente Estrada, Lucia De Franceschi, Maurizio Miano, Antonella Massa, Alessandra Quota, Vincenzo Voi, Monica Fortini, Maria Domenica Cappellini, Inmaculada Fernández-Rozas, Marco Marziali, Giovanna Graziadei, Angelantonio Vitucci, Alberto Piperno, Iván Núñez Gil Md, Irene Motta, Wulandewi Marhaeni, Marco Zecca, Maddalena Casale, Antonio Piga, Silverio Perrotta, Bryan Rupinski, Charbel Maroun, Filomena Longo, Rodolfo Romero, R. Mariani, Domenico Roberti, Susanna Barella, Andrea Beccaria, Valeria Pinto, Barbara Gianesin, Mohammad Abumayyaleh, Rosamaria Rosso, Carolina Espejo Paeres, Álvaro Aparisi, Gian Luca Forni, Rita Gamberini, Ibrahim Akin, Anna Rita Denotti, Federico Bonetti, Alessia Marcon, El-Battrawy, Ibrahim, Longo, Filomena, Núñez Gil, Iván J, Abumayyaleh, Mohammad, Gianesin, Barbara, Estrada, Vicente, Aparisi, Álvaro, Arroyo-Espliguero, Ramón, Balocco, Manuela, Barella, Susanna, Beccaria, Andrea, Bonetti, Federico, Casale, Maddalena, De Michele, Elisa, Denotti, Anna Rita, Fidone, Carmelo, Fortini, Monica, Gamberini, Maria Rita, Graziadei, Giovanna, Lisi, Roberto, Massa, Antonella, Marcon, Alessia, Rubinski, Bryan, Miano, Maurizio, Motta, Irene, Pinto, Valeria Maria, Piperno, Alberto, Mariani, Raffaella, Putti, Maria Caterina, Quota, Alessandra, Ribersani, Michela, Marziali, Marco, Roberti, Domenico, Rosso, Rosamaria, Tartaglione, Immacolata, Vitucci, Angelantonio, Voi, Vincenzo, Zecca, Marco, Romero, Rodolfo, Marouneld, Charbel, Fernández-Rozas, Inmaculada, Espejo, Carolina, Marhaeni, Wulandewi, Garcia Aguado, Marco, Cappellini, Maria Domenica, Perrotta, Silverio, De Franceschi, Lucia, Piga, Antonio, Forni, Gian Luca, and Akin, Ibrahim

Subjects
Male, Pediatrics, medicine.medical_specialty, Iron Overload, Thalassemia, medicine.medical_treatment, Splenectomy, thalassaemia, Disease, law.invention, law, medicine, Humans, Registries, Continuous positive airway pressure, COVID-19, SARS-CoV-2, mortality, business.industry, Acute kidney injury, Cell Biology, medicine.disease, Intensive care unit, Hospitals, Oxygen, Cohort, Molecular Medicine, Female, business, Kidney disease
Abstract

Background: Although numerous patient specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalasemic syndromes in COVID-19 patients remains poorly understood. Aims: We studied the outcomes of 137 COVID-19 patients with a history of Transfusion Dependent Thalassemia (TDT) and non-Transfusion Dependent Thalassemia (NTDT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalasemia. Results: The mean age of thalassemia patients included in our study was 41±16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 25% of thalassemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to the an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassemia group compared to the matched cohort with no history of thalassemia. Amongst thalassemia patients in general, the NTDT group exhibited a higher rate of hospitalization compared to the TDT group (p=0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the NTDT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassemia (either TDT or NTDT) was found to be independently associated with reduced all-cause mortality. Conclusions: The presence of thalassemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models. Trial Registration for sources of data extraction: NCT: 04334291, 04746066 Funding Statement: This study was funded by a non-conditioned grant from FUNDACION INTERHOSPITALARIA PARA LA INVESTIGACION CARDIOVASCULAR, FIC. (Madrid, Spain). This nonprofit institution had no role in the study design, the collection, analysis and interpretation of data, the writing of the report, or the decision to submit the paper for publication. Declaration of Interests: The authors declare no conflicts of interest. Ethics Approval Statement: The study was approved by the Ethics Committees in all of the centers involved (Banjarmasin, Bari, Cagliari, Catania, Ferrara, Gela, Genoa, Getafe, Guadalajara, Legan, Madrid, Mannheim, Milan, Monza, Naples, Olbia, Padua, Pavia, Ragusa, Rome, Salerno, Turin, Valladolid and Verona).

Published
2021
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25. Simultaneous diagnosis of severe SARS-CoV-2 infection and sickle cell disease in two infants.

Author

Parodi E, Voi V, Vania B, Lonardi P, Saracco P, Longobardo A, Grassitelli S, Peruzzi L, Scolfaro C, and Piga A

Subjects
Anemia, Sickle Cell blood, Anemia, Sickle Cell complications, COVID-19 blood, COVID-19 complications, Chromatography, High Pressure Liquid, Female, Fetal Hemoglobin analysis, Hemoglobin, Sickle analysis, Humans, Infant, Male, Anemia, Sickle Cell diagnosis, COVID-19 diagnosis
Published
2021
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26. A prospective randomized controlled trial on the safety and efficacy of alternating deferoxamine and deferiprone in the treatment of iron overload in patients with thalassemia.

Author

Galanello R, Kattamis A, Piga A, Fischer R, Leoni G, Ladis V, Voi V, Lund U, and Tricta F

Subjects
Adolescent, Adult, Deferiprone, Deferoxamine adverse effects, Drug Therapy, Combination, Female, Humans, Male, Pyridones adverse effects, Thalassemia complications, Treatment Outcome, Deferoxamine administration & dosage, Iron Overload drug therapy, Pyridones administration & dosage, Thalassemia drug therapy
Abstract

We compared the safety and efficacy of alternating deferoxamine and deferiprone with that of deferoxamine monotherapy. Sixty transfusion-dependent thalassemia patients regularly treated with deferoxamine were randomized to continue deferoxamine alone or to receive an alternating therapy for one year. Both arms resulted in equivalent decreases of serum ferritin and liver iron concentration. There was no significant difference in the proportion of patients with adverse events in the two therapy groups although the nature of the adverse events differed according to the chelation regimen.

Published
2006

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