Prevalence of SARS-CoV-2 in hemoglobinopathies is modified by age and race.

Prior literature has established a positive association between sickle cell disease and risk of contracting SARS-CoV-2. Data from a cross-sectional study evaluating COVID-19 testing devices (n = 10,567) was used to examine the association between underlying health conditions and SARS-CoV-2 infection in an urban metropolis in the southern United States. Firth's logistic regression was used to fit the model predicting SARS-CoV-2 positivity using vaccine status and different medical conditions commonly associated with COVID-19. Another model using the same method was built using SARS-CoV-2 positivity as the outcome and hemoglobinopathy presence, age (<16 Years vs. ≥16 Years), race/ethnicity and comorbidities, including hemoglobinopathy, as the factors. Our first model showed a significant association between hemoglobinopathy and SARS-CoV-2 infection (OR: 2.28, 95 % CI: (1.17,4.35), P = 0.016). However, in the second model, this association was not maintained (OR: 1.35, 95 % CI: (0.72,2.50), P = 0.344). We conclude that the association between SARS-CoV-2 positivity and presence of hemoglobinopathies like sickle cell disease is confounded by race, age, and comorbidity status. Our results illuminate previous findings by identifying underlying clinical/demographic factors that confound the reported association between hemoglobinopathies and SARS-CoV-2. These findings demonstrate how social determinants of health may influence disease manifestations more than genetics alone.

Hemoglobinopathies and COVID-19: The Experience of a Center in Northern Greece.

Healthcare systems around the globe are still facing the evolving threat of the coronavavirus-19 (COVID-19) pandemic. Hemoglobinopathies include a group of genetic disorders, with the two main entities being thalassemias and sickle cell disease. Due to their immunocompromised status, such patients have been protected as extremely vulnerable to COVID-19 infection. We studied patients with different hemoglobinopathies, consecutively monitored at our center, who were infected with severe acute respiratory syndrome coronavirus 2 (SARS-COV-2) during the second and third waves of the pandemic in Greece (September 2020-April 2021), and associated the outcomes of the infection with the following factors: age, employment, blood type, liver and heart hemosiderosis, splenectomy, concomitant endocrine disorders and transfusion dependency. Among 250 patients monitored at our center, 14 were infected with COVID-19. Nine of them were hospitalized but no one required intensive care unit support and all of them responded to the generally applied treatment plan, despite their comorbidities. Notwithstanding the slightly increased prevalence of COVID-19 in patients with hemoglobinopathies compared to the general population, self-applied measures are still thought to be effective, as our patients got infected through their already sick family members.

From H1N1 to COVID-19: What we have seen in children with hemoglobinopathies.

This work aimed to better understand the impact of pandemics of respiratory viruses on children with hemoglobinopathies through a comprehensive review of the literature. MEDLINE, SCIELO, LILACS, and PUBMED were used as data sources to find articles without time period restrictions. Previous observations suggest that patients with hemoglobinopathies are a group especially susceptible to the complications of viral respiratory infections, with greater morbidity and mortality related to them. Within this context, this review found that, during the 2009 H1N1 pandemic, the risk of hospitalization in children and adults increased, especially in patients with a history of complications such as acute chest syndrome. In addition, the Coronavirus Disease 2019 (COVID-19) pandemic appears to have less repercussion among children with hemoglobinopathies compared to adults, similar to what is seen in the general population. In the H1N1 pandemic, patients with hemoglobinopathies behaved as a group more susceptible to complications, with increased morbidity and mortality. However, for COVID-19, the existing data to date on these patients do not show the same clinical impact. Thus, although these children deserve attention in case of infection due to their potential risks, they seem to have a favorable evolution.

Prevalence of COVID-19 in Egyptian Children With Hemoglobinopathies and Inherited Anemias.

Since the World Health Organization (WHO) announced coronavirus disease-2019 (COVID-19) to be a pandemic, children's COVID-19 cases were generally less severe than adults. The aim of the study was to determine the prevalence of COVID-19 cases among children with hemoglobinopathies and other inherited anemias living in El-Minya Governorate, Egypt, who are at high risk of exposure to infection. This cross-sectional study evaluated data from 258 children with hemoglobinopathies and inherited anemias. A questionnaire was used to collect data about COVID-19 symptoms coupled with appropriate investigations (complete blood count, d-dimer, anti-COVID antibodies, chest computed tomography scans, and polymerase chain reaction). We found 38 of 258 (14.7%) children had mild to moderate COVID-19, while there were no cases with severe form of COVID-19. COVID-19 cases were significantly older (8.63±3.37 vs. 6.71±3.56 y, P =0.01), noncompliant to iron chelators (63.2% vs. 11.8%, P =0.01), had higher serum ferritin (2639.47±835.06 vs. 1038.95±629.87 ng/mL, P <0.0001) and serum iron levels (803.68±261.36 vs. 374.18±156.15 µg/dL, P <0.0001) and more frequently had undergone splenectomy (78.9% vs. 25.5%; P <0.0001) than non-COVID-19 cases. In conclusion, only 14.7% of children with hemoglobinopathies and inherited anemias were recorded to have contracted mild to moderate COVID-19, with no reported severe cases.

Incidence Rate of COVID-19 Infection in Hemoglobinopathies: A Systematic Review and Meta-analysis.

During the coronavirus-19 disease (COVID-19) pandemic, several studies were performed to determine the mortality and incidence rates of coronavirus infection among patients with hemoglobinopathies. However, there has been no systematic approach or meta-analysis to evaluate the results worldwide. This meta-analysis summarized the existing evidence of incidence and mortality rates of COVID-19 and related risk factors among patients with hemoglobinopathies with a focus on ß-thalassemia (ß-thal) and sickle cell disease. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Two authors independently screened the articles, extracted eligible ones, and assessed the quality of studies using the Joanna Briggs Institute (JBI) checklist. The collected data were analyzed by the Stata software. The amount of heterogeneity was demonstrated by the I2 test. The incidence of COVID-19 among patients with a hemoglobinopathy, ß-thal and sickle cell disease was 4.44, 1.34, and 17.22 per 100,000 person-day, respectively, to June 15 2020. The mortality rate of COVID-19 in patients with hemoglobin (Hb) disorders was calculated as 1.07 per 1000 person-day in the same period. Our findings showed a higher incidence rate of COVID-19 in sickle cell disease patients compared to the general population. A slightly higher mortality rate was also observed in patients with hemoglobinopathies compared to the general population, possibly due to the associated risk factors and comorbidities in this vulnerable group, which underscore special care, timely diagnosis and management along with current immunization, were crucial in decreasing the frequency, disease severity and mortality of these patients.

A hypothesis about the role of fetal hemoglobin in COVID-19.

COVID-19 infection is less common in children (with higher fetal hemoglobin levels). In our preliminary study, we also observed a low prevalence and fatality of COVID-19 in countries with high rate of hemoglobinopathy carries. Given these two facts, the hemoglobin structure can play a role in the physiopathology of COVID-19 disease. Several drugs are known to increase fetal hemoglobin in adults. Adding these drugs to COVID-19 clinical trials may improve the patients' outcomes.