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Review
. 2022 Sep;35(3):101382.
doi: 10.1016/j.beha.2022.101382. Epub 2022 Sep 7.

COVID-19 outcomes in sickle cell disease and sickle cell trait

Jana Christian  1 , Sophie Lanzkron  1 , Rakhi P Naik  2
Affiliations
Review

COVID-19 outcomes in sickle cell disease and sickle cell trait

Jana Christian et al. Best Pract Res Clin Haematol. 2022 Sep.

Abstract

Throughout the Coronavirus Disease 2019 (COVID-19) pandemic, understanding the effects of COVID-19 on persons with Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT) has garnered interest. Patients with SCD diagnosed with COVID-19 utilize the emergency department and are hospitalized at significantly higher rates compared to the general population, with vaso-occlusive crisis and acute chest syndrome as the leading presentations. Whether SCD alone increases the likelihood of severe COVID-19 illness remains uncertain; however, potential risk factors for severe disease among patients with SCD include older age, frequent acute care visits for pain, haemoglobin SC disease, and pre-existing end-organ disease. SCT status may also influence COVID-19 outcomes, particularly among those with pre-existing co-morbidities. Corticosteroids in patients with SCD and COVID-19 should be used with extreme caution given strong associations between corticosteroid exposure and severe vaso-occlusive crisis, with prophylactic transfusion administered if corticosteroids are deemed necessary. Hydroxyurea may be protective in COVID-19.

Keywords: Anemia; COVID-19; Sickle cell; Sickle cell trait.

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Conflict of interest statement

Declaration of competing interest S Lanzkron reports consultancy at Bluebird bio, Novo Nordisc, Pfizer, Novartis, GMI; research funding from Imara, Novartis, GBT, Takeda, CSL-Behring, HRSA, PCORI; and stocks with Pfizer and Teva. J Christian and R Naik have no relevant conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
Risk Factors for Severe Illness in SCD and COVID-19: Severe illness in patients with SCD and COVID-19 has been found to be associated with older age (age >40 years old), presence of pre-existing end-organ disease (including heart failure, pulmonary hypertension, chronic kidney disease, and stroke), history of prior acute care visits for pain, and Hb SC disease.
Fig. 2
Fig. 2
Treatment considerations for hospitalized patients with sickle cell disease.
See this image and copyright information in PMC

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