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Editorials
Figure 1. Triage of managing individuals with Sickle cell disease in the COVID-19 pandemic. *Loss of smell (anosmia) and taste (ageusia) and change in taste (dys- geusia) are emerging symptoms. GI symptoms (diarrhea, nausea, vomiting) with or without respiratory symptoms are reported in significant number of COVID-19 patients. **There has been conflicting evidence regarding the utility of antimalarial agents such as hydroxychloroquine17 and the decision to utilize such therapy should take into account potential adverse effects such as ventricular arrythmias and QT prolongation common pre-existing conditions in SCD patients. Importantly, a large majority of Sickle cell disease (SCD) patients are of African descent and are at risk of drug-induced hemolysis due to concomitant G6PD deficiency. Use of hydroxychloro- quine has also been associated with significant methemoglobinemia in case reports.18 With the current lack of evidence on associated risks and complications, registries to capture global information on COVID-19 SCD cases have been established: https://covidsicklecell.org/http://eurobloodnet.eu/news/99/covid-19-infection-and-red- blood-cell-disorders. The sickle cell community can also obtain guidance on the management of individuals with SCD on the ASH website: https://www.hematology.org/covid-19/covid-19-and-sickle-cell-disease. ACS: acute coronary syndrome; CBC: complete blood count; CT: computed tomography.
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on hydroxychloroquine and received exchange transfusion which reduced the Hb S level from 87.1% to 18.1%. He made a full recovery and was discharged after 16 days in the hospital.
The Amsterdam University Medical Centers reported two Hb SS patients who presented with typical acute sick- le pain with no accompanying flu-like symptoms.8 A 24- year-old male presented with acute thoracic pain in the absence of fever or dyspnea; throat and nose swabs were negative for SARS-CoV-2. Computed tomography (CT) imaging revealed bilateral pulmonary infiltrates and the patient received the presumptive diagnosis of a vaso- occlusive crisis (VOC) complicated by ACS. He received antibiotic therapy and was discharged home only to return 24 hours later with increasing pain, dyspnea, and fever. Repeat chest imaging demonstrated progression of infiltrates but radiologic findings were not consistent with COVID-19 pneumonia. A repeat PCR performed on a spu- tum sample was positive for SARS-CoV-2. The patient received appropriate supportive treatment and had an uneventful recovery. Patient 2, a 20-year-old female pre- sented with an acute pain crisis. She developed transient hypoxia and subsequent SARS-CoV-2 testing was posi- tive. Although CT imaging of the chest did not demon- strate pulmonary abnormalities, she was hospitalized for pain management and never developed respiratory symp- toms or fever. We have not been informed if these patients were on hydroxyurea or regular blood transfusion pro- grams.
Although anecdotal, a few important themes emerge
from these cases – that COVID-19 might trigger a VOC without the accompanying respiratory manifestations of COVID-19, that ACS is not as common a complication as feared, and that fever was notably absent at presentation in some SCD patients,6-8 a feature also noted among 5,700 patients admitted with COVID-19 in New York City.9
Are patients with SCD at greater risk for serious illness secondary to COVID-19?
Data from non-SCD cohorts have demonstrated that advanced age and the presence of medical co-morbidities including cardiovascular disease, hypertension, diabetes mellitus, and pre-existing lung disease place individuals at higher risk for developing severe complications as a result of COVID-19, including catastrophic acute hypoxic respira- tory failure.9,10 Critically ill patients can develop a cytokine storm, progressive endothelial activation with associated risk of micro- and macrothrombi, and disseminated intravascular coagulation (DIC) resulting in multi-organ fail- ure. Marked elevations in D-dimer and prothrombin with a reduction in fibrinogen levels herald a worse prognosis and heightened risk of death in COVID-19 patients.11
Patients with SCD, in particular older adults, often have multiple comorbidities with progressive renal insufficiency, hypertension, and chronic lung disease including pul- monary hypertension.12 Viral infection can trigger acute vaso-occlusive crises, including ACS which is associated with high mortality rates.13 In this setting of multi-organ dysfunction, in particular chronic lung damage, COVID-19 could easily trigger ACS and multi-organ failure.
haematologica | 2020; 105(11)