COVID-19 Presentation in Patients with Sickle Cell Disease: A Case Series

Case series Patients: Male, 30-year-old • Male, 30-year-old • Male, 49-year-old • Male, 23-year-old • Female, 25-year-old Final Diagnosis: Sickle cell anemia • sickle cell disease Symptoms: Chest pain • congestion • cough • dyspnea • fever • flank pain • vasoocclusive crisis Medication: — Clinical Procedure: — Specialty: Hematology OBJECTIVE: Rare co-existance of disease or pathology BACKGROUND: Certain health conditions have been proven to have an effect on the severity of COVID-19, the disease caused by SAR-COV-2. The list of identified comorbid conditions includes hematological diseases, with sickle cell disease (SCD) falling into this category. CASE REPORTS: This case series examines the history, presentation, and clinical course of 5 patients with SCD who tested positive for SAR-COV-2 during the spring and summer of 2020. These patients experienced COVID-19 severities ranging from a mild cough and congestion to 8-day hospitalizations requiring blood transfusions. CONCLUSIONS: While there is still a great amount of research on the interaction between COVID-19 and SCD needed, from this study we have concluded that patients with SCD do not always present with the classic COVID-19 triad of cough, shortness of breath, and fever. Often, these patients present with symptoms of vaso-occlusive crisis (VOC), including severe leg, flank, and chest pain, as was seen in 4 of 5 of our patients. We, and several other researchers, believe that this association between COVID-19 and VOC could be due to COVID-19 triggering inflammatory cytokines (notably IL-6) leading to system-wide inflammation, which induces sickling of the red blood cells. Based on this report, we recommend that SCD patients presenting with VOC who have had exposure to SAR-COV-2 be promptly tested for SAR-COV-2 to guide treatment and reduce mortality and morbidity in this vulnerable population.