key: cord-0970622-l273kj0g
authors: Azerad, Marie‐Agnès; Bayoudh, Firas; Weber, Thierry; Minon, Jean‐Marc; Ketelslegers, Olivier; Hoyoux, Marie; Ren, Xueying; Kaye, Olivier; De Marneffe, Nils; Fraipont, Vincent; Masset, Catherine; Bouillon, Anne Sophie; Jaspers, Aurélie; Tebache, Malek; D'Hoen, Guillaume; Habibi, Anousha; Efira, André; Thachil, Jecko; Deckmyn, Hans; Beguin, Yves
title: Sickle cell disease and COVID‐19: Atypical presentations and favorable outcomes
date: 2020-08-04
journal: EJHaem
DOI: 10.1002/jha2.74
sha: 37f75e7226bdb1c2221505671cc0213ce50a960a
doc_id: 970622
cord_uid: l273kj0g

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oped only mild symptoms with few respiratory symptoms at presentation that did not require intensive care unit admission. All three of them initially received automated red cell exchange transfusion (ET) according to the current recommendations [1] .

Our first patient is a young woman with SS Bantu phenotype, aged 23, who came for a regular visit for planning her next ET. Her usual treatment regimen was ET using six red blood cell units (RBCU) every 5 weeks with a target HbS fraction of 50% for previous recurrent painful vaso-occlusive crises (VOC). She was also receiving treatment with hydroxycarbamide but with poor compliance, folic acid, and aspirin to maintain her arteriovenous fistula. She mentioned having had cough 2 weeks prior to the current visit and a fever of 38 • C treated by her general practitioner with amoxicillin for 7 days with good response except for some mild VOC symptoms. She was screened positive for COVID-19 by PCR nasopharyngeal swab. Computed tomography (CT) of her thorax showed unilateral ground glass features suggestive of COVID-19. She was hospitalized for 48 h. ET was performed the following day and also treated with clarithromycin for 7 days with very good outcome and no need for re-hospitalization.

The second patient is a 44 year-old man with SS SCD with very few past episodes of VOC but with history of hypertension and severe renal insufficiency with significant proteinuria. He had basal hemoglobin (Hb) around 5.4 g/dL despite weekly erythropoietin (EPO) administration and poor compliance with low-dose hydroxyurea and ACE inhibitors. He came for a routine visit with a symptomatic leg ulcer for 2 weeks, which he linked to hydroxyurea that he had been more compliant with at a low dose of 500 mg daily. He also mentioned coughing and slight dyspnea for about a week. He was tested SARS-CoV-2 positive by PCR on the nasopharyngeal swab. His chest CT scan showed typical features of COVID infection. After a transfusion of two RBCUs, an isovolumic ET with an HbS target fraction of 20%, and a target Hb of 7 g/dL was also performed. He also received hydroxychloroquine while an inpatient according to our local practice. About 10 days after admission, he developed abdominal pain with ascites and hepatic enlargement. She received no specific COVID-19 treatment except oxygen support and antibiotherapy with ceftriaxone with oxygen dependency initially but improved well and was discharged at day 51. [8] . SCD is due to the modification of a single gene on chromosome 11, which will lead to a loss of membrane deformabil- been used for more than 10 years in Europe and has changed the prognostic of sickle cell patients [15] . Hydroxyurea not only augments both HbF level and red blood cell deformability but also maintains the endothelium and Neutrophils Extracellular Traps (NET) equilibrium, and impacts the release of microparticles [16] . In the French registry, in patients with the worst outcome, age was mentioned as the most relevant risk factor but the mean dosage of Hydroxurea in those patients was also low and could have been a confounding factor [6] . Vitamin D deficiency may be correlated with severity of SARS-Cov 2 infections especially in ethnic minorities [17] , however the vitamin D levels of our Malek Tebache 11

Guillaume D'Hoen 12

American Society of Hematology: COVID-19 and sickle cell disease: frequently asked questions Version 3.0 updated

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Marie-Agnès Azerad https://orcid.org/0000-0001-5094-8738