As for the general population, the presence of co-morbidities such as diabetes, heart disease, pulmonary hypertension, reduced kidney and / or liver function, worsen the effects of the COVID-19 infection in patients affected by red blood cell disorders (RBCDs), especially sickle cell disease (SCD) and Thalassaemia patients. However, this group of patients affected by a chronic life threating disorder that become multi-organic over the time are likely to be at increased risk of complications from COVID-19. Patients at highest risk include the elderly (>50 in our population), those with a history of respiratory or cardiac disease and those with other co-morbidities.
Most of patients affected by RBCDs undergo splenectomy as therapeutic option to improve their level of haemoglobin 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 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.
ERN-EuroBloodNet has established a European collaborative platform to pool information in a collaborative way aiming to quickly identify the impact of COVID19 on patients with red blood cell disorders and to understand the impact of risk factors such as prior disease complications.
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.
Inclusion criteria include both pediatric and adults patients with confirmed COVID19 and affected by a rare anaemia disorder due to a red blood cell defect.
Data set elements include:
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.
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.
Scientific Committee shall be expanded to new members providing data to the registry based on number of patients and consensus by Scientific Committee.