COVID-19 Infection and Acute Pulmonary Embolism in an Adolescent Female With Sickle Cell Disease

Abstract Abstract 3998 Poster Board III-934 Introduction Pulmonary embolism (PE) is a leading cause of morbidity and mortality in the U.S. We and others previously have shown that PE is increasingly detected with increasing use of spiral computerized tomography (CT), at lower severity of illness, suggesting earlier PE diagnosis. Although thrombosis is increased in those with sickle cell trait (Austin, Blood 2007), data are lacking on the prevalence and predictors of PE among individuals with sickle cell disease (SCD), in whom thrombosis may be driven by hemolysis-associated endothelial dysfunction and platelet aggregation. Methods We performed a case-control study to compare demographics, co-morbidity, severity of illness, and mortality in SCD cases with PE and SCD controls without PE, by analyzing statewide discharge data (2001-2006) from the Pennsylvania Health Care Cost Containment Council (PHC4). For this analysis “SCD” included ICD-9 codes for HbSS, HbS/C, HbS/thal, and HbS trait. Clinical and laboratory data were obtained on local SCD cases and controls from the Medical Archival Record System (MARS) at the University of Pittsburgh, using the same ICD-9 codes. Cases were matched by age within 5 years, race, gender, year of admission, and (for PHC4 data only) hospital system. Results The prevalence of PE among SCD admissions, 2001-2006, was 119/ 20,847 (0.57%), increasing over the period by 41.2%, from 0.51% to 0.72%. By comparison, among non-SCD admissions, the prevalence of PE was 66,440/1,416,109 (4.7%), with a similar rate of increase, 54.0%, from 3.78% to 5.82% over the same period. As compared to controls without PE, SCD PE cases had a longer length of stay, 9 vs. 5 days (p=0.0006), greater severity of illness, two highest scores 11.1% vs. 0%, (p=0.0002), and a higher in-hospital mortality rate, 8.7% vs. 0% (p=0.0011). In the local sample (n=14 cases, n=28 controls), the majority of cases were evaluated by spiral CT scans (92.8%). Co-morbidity rates were no greater in cases than controls, including pneumonia (p=0.227), heart failure (p=0.461), coronary symptoms (p=0.464), or stroke (p=0.183). Risk factors for thrombosis were also similar between groups, including estrogen use (p=0.461), obesity (p= 0.763), hyperlipidemia (p=0.106), hypertension (p=0.146), diabetes (p=0.276), smoking (p=0.069), HIV (p=1.000), and HCV (p=0.667). A similar proportion of cases and controls were in crisis, 35.7% each (p=0.888), and there were no differences in the proportion with HbSS, 35.7% vs. 46.4% (p=0.212), HbS/C, 14.3% vs. 0% (p=0.106), HbS/thal, 0% vs. 3.6% (p=0.667), and sickle trait, 50.0% each (p=1.000). Among cases, the degree of anemia (p=0.276), reticulocytosis (p=0.261), WBC (p=0.257) and platelet count (p=0.254) were similar to that of controls. There was no difference between groups in the proportion receiving hydroxyurea (p=0.167), iron chelation therapy (p=1.000), or red cell transfusions (p=0.262). Conclusions Rates of PE are increasing in hospitalized SCD patients in Pennsylvania, with greater severity of illness and higher mortality than in SCD patients without PE. The clinical and laboratory parameters, including severity of hemolysis measured, do not appear to be predictive of PE. Thus, prospective studies are needed to evaluate risk for PE by other markers of disease severity, including tests of platelet activation, tissue factor activity, and hemostatic activation, e.g. thrombospondin, von Willebrand factor, and ADAMTS13. Disclosures: No relevant conflicts of interest to declare.

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Sickle Cell Disease and COVID-19 Infection: A Single-Center Experience

Blood ◽

10.1182/blood-2021-154240 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 985-985

Author(s):

Sindhu Devarashetty ◽

Kimberly Le Blanc ◽

Udhayvir Singh Grewal ◽

Jacqueline Walton ◽

Tabitha Jones ◽

...

Keyword(s):

Pulmonary Embolism ◽

Sickle Cell Disease ◽

Mortality Rate ◽

Sickle Cell ◽

Adult Patients ◽

Living Alone ◽

Morbidity And Mortality ◽

Acute Chest Syndrome ◽

Single Center ◽

Cell Disease

Abstract Background and Objectives: The COVID-19 (CO19) pandemic caused by SARS-CoV-2 remains a significant issue for global health, economics, and society. Several reports have shown that African Americans (AA) have been disproportionately affected by the CO19 pandemic. Limited data have suggested that sickle cell disease (SCD) could be one of the several reasons for higher morbidity and mortality related to CO19 among AA. Recent reports have suggested higher-than-average morbidity and mortality related to CO19 among patients with SCD. We conducted a retrospective, single-institution study in adult patients with SCD who were diagnosed with CO19 infection and their outcomes. Methods: After IRB approval, we conducted a chart review of adult patients (greater than 18 years) with SCD who were diagnosed with CO19 infection between March 1st, 2020, and March 31st, 2021. We recorded demographic data including age, gender, social factors (the type of insurance, availability of primary care provider (PCP), living alone/not), clinical parameters (type of SCD, co-morbidities), outpatient management of SCD, and how CO19 infection was managed like inpatient admission and complications. In patients who were admitted or seen in the emergency department (ED), we collected additional data including vitals, labs, the severity of illness, complications, length of stay, and outcomes. Computations were performed using statistical software SAS 9.4 for Windows. Results: We found a total of 51 patients with SCD diagnosed with CO19 infection in the above period. The median age of patients was 30 years. 61% were females and 39 % were males. All of them were AA. 11.76% were living alone, 49.02% were living with family, 1.96% (1 patient) was institutionalized, and the living situation was unknown in 37.25%. Most of the patients had Medicaid Insurance (52.94%), Medicare in 33.3%, private insurance in 13.73 % and 2% were uninsured. Only 64.71% of patients had a PCP. 60% had HbSS disease, 32% had HbSC disease, 4% had HbS-beta thalassemia, one patient each had HbSS with hereditary persistence of HbF and HbS/HbD. Comorbidities and previous history included acute chest syndrome in 65.96%, avascular necrosis in 36.96%, leg ulcers in 8.7%, hypertension in 8.7%, sickle cell retinopathy in 14.57%, cerebrovascular disease in 26.19%, chronic kidney disease in 7.69%, venous thromboembolism (VTE) in 20.41%, 10.41% were on anticoagulation, history of HIV and hepatitis C infection in 6.38%. 28.21% of patients were maintained on partial exchange transfusions as an outpatient for various indications. 72.73% were on hydroxyurea, 7.5% were on crizanlizumab, 5.26% were on voxelotor and 26.83% were on iron chelation. Vitals and pertinent lab values on initial assessment were recorded and many patients had missing data. On presentation, 25.53% were febrile, 29.17% of patients were tachycardic, 31.25% were hypoxic (SpO2 < 95%), 38.46% were tachypneic, 59.18% had a body mass index (BMI) of > 24.9. Median hemoglobin and hematocrit were 8.9/27.4 g/dL. The median white blood cell count was 9490/uL and platelets were 315,000/uL. Median ferritin was 1573 ug/L. Median bilirubin and creatinine were 2.05 mg/dL and 0.86 mg/dL. The patients were further stratified based on the clinical location where CO19 infection was managed (Table 1). 39.3% were diagnosed in the outpatient setting/ED and 60.3% in the inpatient setting. Among 51 patients, 5.71% (n=2) required ICU admission and was mechanically ventilated. 17.5% received dexamethasone, 7.69% received remdesivir, 2.76% received convalescent plasma, 17.07% had infections and 47% received antibiotics. Only one patient received an exchange transfusion during admission. One patient developed a new VTE after CO19 infection. On statistical analysis, the only factor which impacted the clinical location of management was tachycardia (P=0.007). Of the 51 patients, only 3.9% (2 patients) died of complications of CO19 infection, one with hypoxic respiratory failure, disseminated intravascular coagulation, shock, and the other one with pulmonary embolism. 13% were readmitted within a month, one of them was admitted with a new pulmonary embolism and the others were admitted for acute painful episodes. Conclusion: We found a mortality rate of 3.9% in our single-center study of patients with SCD and CO19 infection. This mortality rate is lower than other published experiences in patients with SCD and CO19 infection. Figure 1 Figure 1. Disclosures Master: Blue Bird Bio: Current holder of individual stocks in a privately-held company.

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