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

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          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. IMAGE 1 A, Chest CT imaging of patient 1 at first presentation to the emergency room (ER) showing infiltrates at basal fields. B, CT imaging at second ER presentation of patient 1, showing an increase in double‐sided infiltrates 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.

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          Clinical course and risk factors for mortality of adult inpatients with COVID-19 in Wuhan, China: a retrospective cohort study

          Summary Background Since December, 2019, Wuhan, China, has experienced an outbreak of coronavirus disease 2019 (COVID-19), caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Epidemiological and clinical characteristics of patients with COVID-19 have been reported but risk factors for mortality and a detailed clinical course of illness, including viral shedding, have not been well described. Methods In this retrospective, multicentre cohort study, we included all adult inpatients (≥18 years old) with laboratory-confirmed COVID-19 from Jinyintan Hospital and Wuhan Pulmonary Hospital (Wuhan, China) who had been discharged or had died by Jan 31, 2020. Demographic, clinical, treatment, and laboratory data, including serial samples for viral RNA detection, were extracted from electronic medical records and compared between survivors and non-survivors. We used univariable and multivariable logistic regression methods to explore the risk factors associated with in-hospital death. Findings 191 patients (135 from Jinyintan Hospital and 56 from Wuhan Pulmonary Hospital) were included in this study, of whom 137 were discharged and 54 died in hospital. 91 (48%) patients had a comorbidity, with hypertension being the most common (58 [30%] patients), followed by diabetes (36 [19%] patients) and coronary heart disease (15 [8%] patients). Multivariable regression showed increasing odds of in-hospital death associated with older age (odds ratio 1·10, 95% CI 1·03–1·17, per year increase; p=0·0043), higher Sequential Organ Failure Assessment (SOFA) score (5·65, 2·61–12·23; p<0·0001), and d-dimer greater than 1 μg/mL (18·42, 2·64–128·55; p=0·0033) on admission. Median duration of viral shedding was 20·0 days (IQR 17·0–24·0) in survivors, but SARS-CoV-2 was detectable until death in non-survivors. The longest observed duration of viral shedding in survivors was 37 days. Interpretation The potential risk factors of older age, high SOFA score, and d-dimer greater than 1 μg/mL could help clinicians to identify patients with poor prognosis at an early stage. Prolonged viral shedding provides the rationale for a strategy of isolation of infected patients and optimal antiviral interventions in the future. Funding Chinese Academy of Medical Sciences Innovation Fund for Medical Sciences; National Science Grant for Distinguished Young Scholars; National Key Research and Development Program of China; The Beijing Science and Technology Project; and Major Projects of National Science and Technology on New Drug Creation and Development.
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            Causes and outcomes of the acute chest syndrome in sickle cell disease. National Acute Chest Syndrome Study Group.

            The acute chest syndrome is the leading cause of death among patients with sickle cell disease. Since its cause is largely unknown, therapy is supportive. Pilot studies with improved diagnostic techniques suggest that infection and fat embolism are underdiagnosed in patients with the syndrome. In a 30-center study, we analyzed 671 episodes of the acute chest syndrome in 538 patients with sickle cell disease to determine the cause, outcome, and response to therapy. We evaluated a treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy. Samples of blood and respiratory tract secretions were sent to central laboratories for antibody testing, culture, DNA testing, and histopathological analyses. Nearly half the patients were initially admitted for another reason, mainly pain. When the acute chest syndrome was diagnosed, patients had hypoxia, decreasing hemoglobin values, and progressive multilobar pneumonia. The mean length of hospitalization was 10.5 days. Thirteen percent of patients required mechanical ventilation, and 3 percent died. Patients who were 20 or more years of age had a more severe course than those who were younger. Neurologic events occurred in 11 percent of patients, among whom 46 percent had respiratory failure. Treatment with phenotypically matched transfusions improved oxygenation, with a 1 percent rate of alloimmunization. One fifth of the patients who were treated with bronchodilators had clinical improvement. Eighty-one percent of patients who required mechanical ventilation recovered. A specific cause of the acute chest syndrome was identified in 38 percent of all episodes and 70 percent of episodes with complete data. Among the specific causes were pulmonary fat embolism and 27 different infectious pathogens. Eighteen patients died, and the most common causes of death were pulmonary emboli and infectious bronchopneumonia. Infection was a contributing factor in 56 percent of the deaths. Among patients with sickle cell disease, the acute chest syndrome is commonly precipitated by fat embolism and infection, especially community-acquired pneumonia. Among older patients and those with neurologic symptoms, the syndrome often progresses to respiratory failure. Treatment with transfusions and bronchodilators improves oxygenation, and with aggressive treatment, most patients who have respiratory failure recover.
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              Acute chest syndrome in sickle cell disease due to the new influenza A (H1N1) virus infection.

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                Author and article information

                Contributors
                e.nur@amc.uva.nl
                Journal
                Am J Hematol
                Am. J. Hematol
                10.1002/(ISSN)1096-8652
                AJH
                American Journal of Hematology
                John Wiley & Sons, Inc. (Hoboken, USA )
                0361-8609
                1096-8652
                21 April 2020
                June 2020
                : 95
                : 6 ( doiID: 10.1002/ajh.v95.6 )
                : 725-726
                Affiliations
                [ 1 ] Department of Clinical Hematology Amsterdam University Medical Centers Amsterdam The Netherlands
                Author notes
                [*] [* ] Correspondence

                Erfan Nur, Department of Clinical Hematology, Amsterdam University Medical Centers, Amsterdam, The Netherlands.

                Email: e.nur@ 123456amc.uva.nl

                Author information
                https://orcid.org/0000-0002-7069-930X
                https://orcid.org/0000-0002-7534-5084
                Article
                AJH25821
                10.1002/ajh.25821
                7262303
                32267016
                6bf1db12-dfb6-434a-bab5-f3b330e06fbd
                © 2020 The Authors. American Journal of Hematology published by Wiley Periodicals, Inc.

                This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                : 31 March 2020
                : 02 April 2020
                : 03 April 2020
                Page count
                Figures: 1, Tables: 0, Pages: 2, Words: 913
                Categories
                Diagnostic Imaging in Hematology
                Clinical Pearls in Blood Diseases
                Diagnostic Imaging in Hematology
                Custom metadata
                2.0
                June 2020
                Converter:WILEY_ML3GV2_TO_JATSPMC version:5.8.3 mode:remove_FC converted:01.06.2020

                Hematology
                Hematology

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