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778 COVID 19 and Hemoglobinopathies: Update of the Italian Experience

Program: Oral and Poster Abstracts
Session: 112. Thalassemia and Globin Gene Regulation: Poster I
Hematology Disease Topics & Pathways:
Coronaviruses, SARS-CoV-2/COVID-19, sickle cell disease, Diseases, thalassemia, Hemoglobinopathies
Saturday, December 5, 2020, 7:00 AM-3:30 PM

Irene Motta, MD1,2*, Valeria Maria Pinto, MD3*, Filomena Longo, MD4*, Federico Bonetti, MD5*, Elisa De Michele, MD6*, Alberto Piperno, MD7*, Lorella Pitrolo, MD8*, Micol Quaresima, MD9*, Michela Ribersani, MD10*, Margherita Migone De Amicis, MD2*, Manuela Balocco, MD3*, Barbara Gianesin, PhD11*, Giovanna Graziadei, MD2*, Maria Domenica Cappellini, MD1, Lucia De Franceschi, MD12, Antonio Piga, MD4 and Gian Luca Forni, MD3*

1Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
2Department of Internal Medicine, UOC Medicina Generale, Foundation IRCCS Ca' Granda, Ospedale Maggiore Policlinico, Milan, Italy
3Hemoglobinopathies and Congenital Anemia Center, Ospedale Galliera, Genoa, Italy
4Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
5Pediatric Haematology Oncology, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy
6Medicina Trasfusionale, AUO "San Giovanni di Dio e Ruggi D'Aragona", Salerno, Italy
7Centre for Disorder of Iron Metabolism, ASST-Monza - S.Gerardo Hospital, Monza, Italy
8Campus of Hematology Franco and Piera Cutino, AOOR Villa Sofia-V. Cervello, Palermo, Italy
9Dipartimento di Medicina Diagnostica, Clinica e di Sanità Pubblica, University of Modena and Reggio Emilia, Modena, Italy
10UOC Haematology, Azienda Ospedaliera Universitaria Policlinico Umberto I di Roma, Rome, Italy
11ForAnemia Foundation, Genoa, Italy
12Department of Medicine, Policlinico GB Rossi, University of Verona, Verona, Italy

Background. Patients with pre-existent chronic morbidities are likely to be more severely affected by SARS-Cov2 infection. In Italy, the “Società Italiana Talassemie ed Emoglobinopatie” (SITE) has recently estimated the number of patients (Pts) with Hemoglobinopathies followed by Italian Specialized Centers (SITE Network). Five thousand Transfusion-dependent beta-thalassemia (TDT), 1900 Non-Transfusion-dependent beta-thalassemia (NTDT) and 2000 Sickle Cell Disease (SCD) were registered [1]. To verify the impact of SARS-CoV-2 infection on Pts with Hemoglobinopathies, we performed a specific survey by electronic Case Report Form (eCRF). Inclusion criteria included positive swab or serology in a patient with hemoglobinopathy and at least 15 days of follow-up from either the onset of symptoms or SARS-CoV2 positivity. The survey was approved by the Ethics Committee, and eCRF was shared with the Centers of Italian Hemoglobinopathies Network. Preliminary data updated to April 10, 2020, were published [2].

Results. As of July 31, 2020, 27 cases have been reported: 18 TDT, 4 NTDT, 5 SCD. 89% of the cases were in Northern Italy, where the rate of infection was much higher than the rest of the country, reflecting the national epidemiology. The mean age of thalassemia patients (TDT and NTDT) was 43±11 years, and 55% were male; the mean age of SCD patients was 33±15 years, and 40% was male. The likely source of infection has been detected in 63% (17/27) of cases: 11 had occupational exposure, 6 had a positive relative. Five patients were asymptomatic: for them, the SARS-CoV-2 infection was identified by positive swab for 1 patient and by positive level of IgG for 4. Twenty patients had associated comorbidities, 14 were splenectomized, and 3 had functional asplenia. Eleven patients were hospitalized, only one in high-intensity care unit. Three patients required more intensive ventilation support with continuous positive airway pressure (CPAP), one of these has a history of diffuse large B-cell lymphoma treated with chemotherapy in the previous year. Three other patients required support by oxygen. No Pts required intubation. Two Pts increased blood requirement. Only five received supposedly specific treatment for COVID-19: two hydroxychloroquine (HCQ), one HCQ plus ritonavir/darunavir, and one HCQ plus anakinra, one HCQ plus Tocilizumab plus Lopinavir/Ritonavir. The clinical course of hospitalized patients was 18±7 days. All patients recovered.

Conclusions. The prevalence of COVID-19 infection in Italian patients with Hemoglobinopathies result 0,3% while in general population the prevalence in Italy is 0,4% [3]. Considering that the thalassemia population is more strictly observed, we could postulate that the precautions suggested or self-applied by the Pts were effective. No death nor severe SARS with intubation, nor signs of cytokines storm, only one thromboembolic event was observed although most individuals had pre-existing complications. A single case with pulmonary hypertension has been described in detail [4]. In most individuals the infection has been pauci or asymptomatic and all recovered. This experience differs from what has been observed in Iran on a similar series with different severity and mortality and ask for a more in-depth comparison [5]. In conclusion, our data do not indicate increased severity of COVID-19 in Pts with Hemoglobinopathies followed in Specialized Centers.

Acknowledgment. We would like to thank ALT (Associazione per la Lotta alla Talassemia R.Vullo - Ferrara)..


  1. http://www.site-italia.org/2020/covid-19.php. SITE communication. Accessed April 1, 2020
  2. Motta I, Migone De Amicis M, Pinto VM, et al. SARS-CoV-2 infection in beta thalassemia: Preliminary data from the Italian experience. Am J Hematol. 2020;95(8): E198-E199.
  3. https://www.epicentro.iss.it/coronavirus/sars-cov-2-dashboard, Accessed July 31, 2020
  4. Pinto VM, Derchi GE, Bacigalupo L, Pontali E, Forni GL. COVID-19 in a Patient with β-Thalassemia Major and Severe Pulmonary Arterial Hypertension. Hemoglobin. 2020;44(3):218-220.
  5. Karimi M, Haghpanah S, Azarkeivan A, et al. Prevalence and mortality in β-thalassaemias due to outbreak of novel coronavirus disease (COVID-19): the nationwide Iranian experience. Br J Haematol. 2020;190(3):e137-e140.

Disclosures: Motta: Sanofi Genzyme: Honoraria. Cappellini: BMS: Honoraria; Genzyme/Sanofi: Honoraria, Membership on an entity's Board of Directors or advisory committees; CRISPR Therapeutics, Novartis, Vifor Pharma: Membership on an entity's Board of Directors or advisory committees. Piga: BMS: Research Funding; Novartis: Research Funding. Forni: Novartis: Membership on an entity's Board of Directors or advisory committees.

*signifies non-member of ASH