Sickle cell disease and COVID‐19: Susceptibility and severity

Abstract

We surveyed published papers and an international sickle cell disease (SCD) registry to detect susceptibility and clinical course of coronavirus disease 2019 (COVID‐19) in SCD patients. COVID‐19 presentation was mild in children and moderate in many SCD adults. Regarding increased comorbidities with age, it seems severe COVID‐19 to be more common in older SCD patients. Although the overall outcome of COVID‐19 was favorable in SCD children, a high rate of pediatric intensive care unit admission should be considered in managing these patients. To explain COVID‐19 outcome in SCD patients, the possible benefits of hydroxyurea therapy could be considered. The obtained results should be interpreted, considering low cases from sub‐Saharan people, younger age of SCD patients compared to general population, a bias toward registry of the more severe form of disease, the effect of pre‐existing comorbidities with multisystem organ damage, and the role of health socio‐economic determinants.

Prognostic factors associated with COVID-19 related severity in sickle cell disease

“…Also notable however, as reported previously [8] , [21] , is that a majority of hospitalizations were for SCD-related rather than COVID-19 related complications, most frequently acute painful episodes. In fact, pediatric patients had no COVID-19 related complications, consistent with previously published milder courses in pediatric SCD patients [3] , [5] , [6] , [9] . Our cohort mortality of 2.5% is on the low end of the 3–10% range in SCD previously reported [1] , [4] , [5] , [21] ; higher than the U.S. general population symptomatic fatality of 1.1% [22] but perhaps not higher when specifically compared to a U.S. black population [4] , [8] .…”

“…Despite possible increased COVID-19 disease severity in SCD, one large study did not show an increased fatality rate in SCD [4] . Patient variables associated with increased clinical severity of COVID-19 infection in SCD patients include older age [1] , [9] , comorbidities [1] , [8] , [9] , [10] , not being on hydroxyurea (HU) [1] , [9] , history of stroke [2] , [8] and black race [5] . Non-SCD studies show similar risk factors such as older age [11] , [12] , comorbidities [11] , [12] , as well as being a health care worker (HCW) [13] , [14] .…”

“…Possible laboratory-based predictors of hospitalization include the following admission values: high C-reactive protein (CRP) [1] , low Hb [1] , [15] , [16] , low lymphocyte count [1] , [15] , high alanine aminotransferase [1] , increased neutrophil-to-lymphocyte ratio [1] , [15] , high immature granulocyte count (promyelocytes, myelocytes and metamyelocytes) [11] , [15] , [17] , high creatinine [1] , and high direct bilirubin [1] . Possible in-hospital mortality values include high D-dimer [1] , [11] , high creatinine levels [1] , [9] , [11] , high lactate dehydrogenase [1] , [11] , and high CRP [11] on admission. Fewer comorbidities may contribute to the better outcome of pediatric patients with COVID-19 infection [9] .…”

“…Possible in-hospital mortality values include high D-dimer [1] , [11] , high creatinine levels [1] , [9] , [11] , high lactate dehydrogenase [1] , [11] , and high CRP [11] on admission. Fewer comorbidities may contribute to the better outcome of pediatric patients with COVID-19 infection [9] . In order to add our experience in the prognosis of SCD patients with COVID-19, we analyzed the clinical course and potential variables associated with need for hospitalization and ICU admission in all SCD patients diagnosed with COVID-19 infection throughout the Northwell Health system between March 1 and Dec 31, 2020.…”

Thalassemia and COVID-19: Susceptibility and Severity

“…Among sickle cell disease (SCD) patients (including sickle/β-thalassemia with COVID-19), the benefit of early simple/exchange transfusion in those patients with acute chest syndrome has been indicated with rapid clinical improvement of tachypnea/dyspnea and oxygen saturation and the clinical course of the disease (14). Also, the mortality rate of non-transfusion-dependent thalassemia (NTDT) patients was higher than transfusion-dependent thalassemia (TDT) patients (15).…”