Clinical predictors of poor outcomes in patients with sickle cell disease and COVID-19 infection

Author:

Minniti Caterina P.1ORCID,Zaidi Ahmar U.2ORCID,Nouraie Mehdi3ORCID,Manwani Deepa4,Crouch Gary D.5,Crouch Andrew S.1,Callaghan Michael U.2,Carpenter Sarah6,Jacobs Charleen7ORCID,Han Jin8,Simon Jena7,Glassberg Jeffrey7,Gordeuk Victor R.9ORCID,Klings Elizabeth S.10

Affiliation:

1. Division of Hematology, Montefiore Health Systems, Albert Einstein College of Medicine, Bronx, NY;

2. Division of Hematology/Oncology, Children’s Hospital of Michigan/Central Michigan University, Detroit, MI;

3. Division of Pulmonary, Allergy and Critical Care Medicine, University of Pittsburgh, Pittsburgh, PA;

4. Division of Pediatric Hematology, Montefiore Health Systems, Albert Einstein College of Medicine, Bronx, NY;

5. Division of Pediatric Hematology/Oncology, Department of Pediatrics, Icahn School of Medicine at Mount Sinai, New York, NY;

6. Department of Emergency Medicine, Boston University School of Medicine, Boston, MA;

7. Department of Emergency Medicine, Icahn School of Medicine at Mount Sinai, New York, NY;

8. Center for Pharmacoepidemiology and Pharmacoeconomic Research, College of Pharmacy, and

9. Division of Hematology and Oncology, Department of Medicine, University of Illinois at Chicago, Chicago, IL; and

10. The Pulmonary Center, Boston University School of Medicine, Boston, MA

Abstract

Abstract We aimed to identify predictors of outcomes and survival in patients living in 4 major metropolitan areas who had sickle cell disease (SCD) and COVID-19 to inform best approaches to prevention and care. Data were collected at baseline and during the clinical course in SCD patients diagnosed with COVID-19 in four COVID-19 epicenters. Patients were followed up posthospital discharge for up to 3 months. Of sixty-six SCD patients with COVID-19, fifty patients (75%) required hospitalization, and seven died (10.6%). Patients with preexisting kidney disease (chronic kidney disease) were more likely to be hospitalized. The most common presenting symptom was vaso-occlusive pain. Acute chest syndrome occurred in 30 (60%) of the 50 hospitalized patients and in all who died. Older age and histories of pulmonary hypertension, congestive heart failure, chronic kidney disease, and stroke were more prevalent in patients who died, as were higher creatinine, lactate dehydrogenase, and D-dimer levels. Anticoagulation use while inpatient was twice less common in patients who died. All deaths occurred in individuals not taking hydroxyurea or any other SCD-modifying therapy. Patients with SCD and COVID-19 exhibited a broad range of disease severity. We cannot definitively state that the overall mortality is higher in patients with SCD, although our case fatality rate was ∼10% compared with ∼3% in the general population, despite a median age of 34 years. Individuals with SCD aged >50 years, with preexisting cardiopulmonary, renal disease, and/or stroke not receiving hydroxyurea, who present with high serum creatinine, lactate dehydrogenase, and D-dimer levels, are at higher risk of death, irrespective of genotype or sex.

Publisher

American Society of Hematology

Subject

Hematology

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