Case Presentation: A 33-year-old woman with sickle cell SC disease (HbSC) was admitted to the hospital for respiratory support after testing positive for COVID-19. Four days prior to presentation, the patient developed subjective fever, cough, shortness of breath, substernal non-radiating chest pain, thigh and back pain. Her medical history included prior vaso-occlusive episodes, though no history of acute chest syndrome. She reported compliance with prophylactic folic acid, penicillin, and as needed hydrocodone.Crackles were present on auscultation. There was no tenderness to palpation over the chest wall or extremities. The patient was afebrile, with blood pressure of 132/86, pulse of 86 and respiration rate of 14.LDH of 392 U/L, c-reactive protein of 2.88 mg/dL, troponin less than 0.015 ng/mL, ferritin of 1167.8 ng/mL, d-dimer less than 2 mg/L and procalcitonin of 0.28 ng/mL. Reticulocyte percent was 1.5%, total bilirubin 1.9 mg/dL and hemoglobin 10 g/dL from a baseline of 12 g/dL. There was minimal sickling on peripheral blood smear.Initial chest X-ray demonstrated patchy mid to lower lung opacities bilaterally (Figure 1).Prophylactic enoxaparin, analgesics for pain, and empiric azithromycin and ceftriaxone were initiated.She developed a fever of 103.0°F and required oxygen supplementation of 3L nasal cannula. Blood cultures from admission returned with no growth, though sputum cultures revealed moderate polymorphonuclear leukocytes and gram-negative rods. Dexamethasone and Remdesivir were added to her treatment regimen.On hospital day 9, pain was resolving and she was weaned off of supplemental oxygen. Repeat chest X-ray demonstrated improvement of bilateral infiltrates (Figure 2).
Discussion: Features of both COVID infection and acute chest syndrome were evident in this patient. The infiltrates on chest x-ray, fever, shortness of breath, chest pain and new oxygen requirement all met the criteria for acute chest syndrome, though also feature in COVID infection. Given the increased rate of mortality in acute chest syndrome, empiric antibiotics and analgesics were continued throughout the patient’s course. Deteriorating clinical status would have prompted more aggressive treatment, including possible transfusion.Also concerning in this hospital course was the initial categorization of sickle cell patients as high risk for complications related to COVID. This stems from a known upregulation of cytokine IL-6 and an increased general inflammatory state, potentially making sickle cell patients more susceptible to severe symptoms. Moreover, sickle cell patients are chronically immunocompromised and have a higher prevalence of thrombo-occlusive events, an issue well documented in COVID infection.
Conclusions: Paradoxically, sickle cell patients with COVID have consistently better outcomes than the general population. A limited review of current case reports and case series of COVID infections in sickle cell patients showed only 5 of 146 (3.4%) deaths with limited numbers of patients requiring intense respiratory intervention. The majority of cases were mild and successfully treated. This is in alignment with our patient’s case, who had a relatively uncomplicated hospital course with eventual discharge. Though a limited sample size, these data serve as an early indication that sickle cell disease patients may have an overall decreased mortality rate compared to the general population.
