Clinical Spectrum of Sickle Cell Disease and COVID-19: Laboratory and Clinical Factors Associated with Morbidity and Mortality (preprint)

Background: The COVID-19 pandemic has generated concern as a potential remarkably severe threat to the sickle cell disease (SCD) population. We aim to identify predictors of outcomes and survival in a large US-based SCD and COVID-19 cohort to inform best approaches to prevention and care. Methods: Clinical data were collected at baseline and during the clinical course using a standardized form in SCD patients diagnosed with COVID-19 at 5 academic centers in four COVID-19 epicenters. Patients were followed post-hospital discharge for up to 3 months. Results: Of 66 consecutive SCD patients with COVID-19, 75% required hospitalization, with a median length of stay of 6 days, and 7 died (10.6%). Patients with preexisting kidney disease were more likely to be hospitalized, while age, sex and genotype had no effect. The most common presenting symptom was vaso-occlusive pain. Chest X-ray was abnormal (acute chest syndrome) at presentation in 30 of 50 (61%) hospitalized and all deceased patients. Older age (median of 53 versus 32 years) and histories of pulmonary hypertension, congestive heart failure and/or stroke were more prevalent in deceased patients, as were high creatinine, lactate dehydrogenase, C-reactive protein, and D-dimers. The use of anti-coagulation, but not hydroxychloroquine or transfusion, during inpatient hospitalization was associated with decreased mortality (p<0.05). All deaths occurred in individuals not taking hydroxyurea or other SCD modifying therapy. Conclusions: Patients with SCD and COVID-19 infection demonstrated a broad range of disease severity, from mild to very severe. COVID-19 in SCD individuals with pre-existing cardiopulmonary, renal disease and/or stroke presenting with pain and high creatinine should be considered at risk of death, irrespective of genotype or gender. Inpatient use of anticoagulation should be considered for all SCD patients with COVID-19. Though older individuals with vasculopathic comorbidities and high D-dimers were more likely to die, the median age of death was decades lower than the non-SCD population. Funding Statement: None. Declaration of Interests: None Ethics Approval Statement: Each respective Institutional Review Board approved data collection as minimal-risk research and waived the requirement for informed consent.