ERN-EuroBloodNet Registry on Patients With Rare Red Blood Cell Defects and COVID-19

Alpha-Thalassemia, Beta-Thalassemia, Beta Thalassemia Intermedia, Beta Thalassemia Major, Delta-Beta Thalassaemia, Dominant Beta-Thalassemia, Elliptocytosis, Hereditary, Enzyme Disorder; Anemia, Haemoglobin C-Beta-Thalassaemia Syndrome, Hemoglobin Barts Hydrops, Hemoglobin E-Beta Thalassemia, Hemoglobin H Disease, Hemoglobinopathies, Hereditary Persistence of Fetal Hemoglobin Thalassemia, Membranes; Disorder, Sickle Beta Thalassemia, Sickle Cell Anemia, Sickle Cell Disease, Sickle Cell Hemoglobin C, Sickle Cell Hemoglobin D, Sickle Cell-Hemoglobin E Disease, Sickle-Cell; Hemoglobinopathy, Spherocytosis, Hereditary, Stomatocytosis

COVID-19, Hemoglobinopathy, Sickle cell disease, Sickle Cell Anemia, Sickle cell-beta-thalassemia, Sickle cell-hemoglobin, Beta-Thalassemia, Beta Thalassemia Major, Beta Thalassemia Intermedia, Delta-Beta Thalassaemia, Alpha-Thalassemia, Hemoglobin, Rare hemolytic anemia, Hereditary Spherocytosis, Hereditary Elliptocytosis, Hereditary Stomatocytosis

Patients with red blood cell disorders (RBCDs), such as Sickle cell disease (SCD) and Thalassemia, are chronic, life-threatening conditions that can become multi-organ complications over time, and are likely at an increased risk of COVID-19-related complications. Patients at the highest risk include the elderly (\>50 in our population), those with a history of respiratory or cardiac disease and those with other comorbidities. Several patients affected by RBCDs undergo splenectomy as therapeutic option to improve their level of hemoglobin concentration. Splenectomized patients, or in the case of SCD with functional hyposplenism, are more vulnerable to bacterial infections / superinfections after viral infection. Acute pulmonary syndrome (ACS) is the main cause of morbidity in SCD in middle-high income countries, and is often triggered by infectious events. Currently, there is no literature on the subject. Thus, any recommendation available comes from the experience gained with previous Coronaviruses infections. Accordingly, the correct treatment and management of infection by Coronavirus SARS-COV-2 (COVID-19) in patients affected by RBCDs may be challenging given the rapid spread of the pandemic and limited literature so far, especially in some countries. Accordingly, there is an urgent need to pool evidence in a unique repository on patients affected by RBCDs and COVID-19 in order to reach critical numbers to facilitate the medical decision making process across Europe. The Registry on patients with rare RBCDs and COVID-19 is an initiative conceived in the core of the European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet, FPA 739541, www.eurobloodnet.eu) aiming at supporting medical practice of COVID-19 in these patients by gathering evidence on pediatric and adult COVID-19 confirmed cases in RBCDs across Europe.

The Registry on patients with rare red blood cell disorders (RBCDs) and COVID-19 is an initiative conceived in the core of the European Reference Network on Rare Hematological Diseases (ERN-EuroBloodNet, FPA 739541, www.eurobloodnet.eu) aiming at supporting medical practice of COVID-19 in these patients by gathering evidence on pediatric and adult COVID-19 confirmed cases in RBCDs across Europe. Primary objective of the registry is to pool evidence on the clinical management and outcomes of patients affected by red blood cell disorders and COVID-19 for supporting daily medical practice while enabling inter-professional consultation of complex cases. Secondary objective includes the performance of observational studies in the different cohorts of patients, including Sickle Cell Disease, Thalassaemia, Enzymopathies and Membranopathies patients in pediatric and or adult stages. The network of hospitals that will be created from this registry will hold regular meetings to analyze the data that are being introduced and to discuss possible measures against COVID-19 based on them. The collaboration will continue with the development of observational studies that will give the necessary evidence to make recommendations for COVID-19 management in hematological patients. METHODOLOGY Inclusion criteria include both pediatric and adults patients with confirmed COVID-19 and affected by a rare anaemia disorder due to a red blood cell defect. Data set elements include: * Demographics: country of living, sex, age (only year of birth) * Data related to Red blood cell disorder: diagnosis, co-morbidities, treatments, splenectomy, blood transfusion requirement. * Data related to COVID-19: date and method for diagnosis, severity grade, clinical manifestations i.e. pneumonia, symptoms days, acute events, treatments, days of hospitalization, days at intensive care unit, sequela, death. The registry has been developed by Vall d'Hebron Research Institute using Redcap, a secure web application for building and managing online databases. Individual patients' data will be gathered in a codified way. ETHICS AND GDPR COMPLIANCE The processing of personal data is conducted fully respecting the Regulation (EU) 2016/679 (General Data Protection Regulation), including legal basis and special requirements and safeguards to ensure the safety and the confidentiality of the data subjects. The Research Ethics Committee of the Vall d'Hebron's Hospital has confirmed that this exceptional case justifies the waiver of informed consent. PROCESSING OF THE DATA The patient's information included in the database is pseudonymised by single codification. The minimization principle of data protection is followed (i.e. only year of age is collected, no identified data is collected, and only clinical data of the health care is collected). The medical doctors at each center have a coding table in which the code can be linked to the patient's personal information. This table is safely guarded by the medical doctor and never leaves the center. All the information stored in the database is pseudonymised.

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