Vaso‐occlusive crisis and acute chest syndrome in sickle cell disease due to 2019 novel coronavirus disease (<scp>COVID</scp>‐19)

Abstract

In March 2020, during the 2019 novel coronavirus disease (COVID-19) pandemic, caused by the newly emerged virus SARS-CoV-2, two patients with homozygous sickle cell disease (SCD) were admitted to our hospital with a painful vaso-occlusive crisis (VOC) triggered by COVID-19. Both patients had no flu-like complaints characteristic of COVID-19 during or preceding the VOC episode. Patient 1, a 24-year-old man with a previous medical history of minor pain episodes without indication for hospitalization, presented with severe right thoracic pain for 3 days. At presentation he had a temperature of 37.6°C; pulse of 76/minute, blood pressure of 106/65 mmHg; respiration rate of 18/min and a peripheral oxygen saturation (SpO2) of 97%. A non-contrast chest CT showed double-sided infiltrates without ground-glass opacities or crazy paving and was not characteristic of COVID-19 (Image 1A). Throat and nose swabs were negative for SARS-CoV-2. A diagnosis of VOC complicated by acute chest syndrome (ACS) was made. Treatment with oxygen, intravenous morphine with patient-controlled analgesia (PCA), fluid replacement therapy and amoxicillin/clavulanic acid was initiated. After 1 day, the level of pain had decreased significantly (numeric rating scale decreasing from nine to two) and the patient remained respiratory stable throughout his hospital stay. He was discharged with amoxicillin/clavulanic acid continued at home. However, the next day he returned to the emergency room with increased pain, dyspnea, respiration rate of 20/minutes, SpO2 of 93% and a temperature of 38.9°C. Chest CT imaging showed progression of the double-sided infiltrates in the lower lobes of the lungs (Image 1B). A second PCR, this time of a sputum sample, was positive for SARS-CoV-2. He was treated with oxygen (up to 5 L/min) and morphine (PCA) while amoxicillin/clavulanic was continued. The patient had a smooth recovery, without a need for exchange transfusion. He was discharged again after 3 days and was instructed to stay at home (in isolation) until 24 hours after becoming completely symptom-free. Patient 2, a 20-year-old woman with a history of frequent VOCs presented with severe pain of the back and extremities for 1 day. She had no respiratory or gastro-intestinal complaints. Because of a dip in SpO2 to 88% after a 100 μg parenteral fentanyl bolus in the ambulance, the suspicion of COVID-19 rose which led to performing a chest CT and a throat and nose swab for a SARS-CoV-2 PCR. While the CT imaging did not show any pulmonary abnormalities, the PCR was positive for SARS-CoV-2. She remained hospitalized to treat her VOC without developing any respiratory symptoms. The World Health Organization (WHO) recently declared SARS-CoV-2 infection a pandemic. Severe respiratory illness occurs in approximately 15%-20% of infected patients.1 As of March 31, 2020, 800.049 laboratory-confirmed cases and 38.714 deaths have been documented globally.2 In SCD, COVID-19 can potentially cause severe (pulmonary) complications, either by directly causing severe pneumonia or by triggering a VOC and/or ACS. While further experience regarding the clinical presentation of COVID-19 in SCD needs to be awaited, the following important points need to be taken into consideration based on the above described patients. Similar to what we know from other viral infections,3, 4 SARS-CoV-2 can also cause ACS in SCD. Furthermore, as can be seen in patient 1, an ACS can develop without the typical pulmonary complications that can be seen with COVID-19. Both patient 1 and especially patient 2 illustrate that COVID-19 might trigger a VOC without the presence of flu-like symptoms of COVID-19. With respect to diagnosis, the history of patient 1 illustrates the low sensitivity of the PCR of the throat and nose swabs in the primary diagnosis of COVID-19, which is estimated to be around 70%. We therefore suggest to perform a second PCR, preferably on a sputum sample, and non-contrast chest CT imaging when there is no alternative explanation for VOC or when the clinical suspicion for COVID-19 remains high. Based on these two patients, at our center we decided to include SARS-CoV-2 PCR in the evaluation of SCD patients presenting with VOC.