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 Table of Contents  
Year : 2020  |  Volume : 11  |  Issue : 2  |  Page : 89-90

Sickle cell disease: High risk or no risk for coronavirus disease 2019 infection

1 Consultant Pediatric Hematologist Oncologist, Department of Hematology, Hereditary Blood Diseases Center Al Ahssa, AlAhssa, Saudi Arabia
2 Consultant Pulmonologist, Department of Respiratory Medicine, King Fahad Hospital, AlAhssa, Saudi Arabia

Date of Submission08-Jun-2020
Date of Decision10-Jun-2020
Date of Acceptance11-Jun-2020
Date of Web Publication28-Jul-2020

Correspondence Address:
Dr. Muneer Hassan Albagshi
Hereditary Blood Disorders Center, Al Ahssa
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joah.joah_95_20

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How to cite this article:
Albagshi MH, Albagshi MH. Sickle cell disease: High risk or no risk for coronavirus disease 2019 infection. J Appl Hematol 2020;11:89-90

How to cite this URL:
Albagshi MH, Albagshi MH. Sickle cell disease: High risk or no risk for coronavirus disease 2019 infection. J Appl Hematol [serial online] 2020 [cited 2023 Jun 4];11:89-90. Available from: https://www.jahjournal.org/text.asp?2020/11/2/89/290971

Coronaviruses (CoVs) are respiratory viruses that caused three outbreaks in this century, starting with severe acute respiratory syndrome (SARS) in 2003, then Middle East Respiratory Syndrome CoV disease in 2012, and CoV disease 2019 (COVID-19) which is the cause of the current pandemic since December 2019. It affected over 7 million and the fatality of around 400,000 up to date. The most vulnerable population is older patients and patients with chronic conditions (lung, heart, and diabetes mellitus).

Sickle cell disease (SCD) is the most important hemoglobinopathy affecting millions around the globe.[1] It is considered an immunocompromised status, which makes these patients vulnerable, because of the pulmonary complications such as acute chest syndrome (ACS), pulmonary embolism, and pulmonary hypertension. In the eastern province of Saudi Arabia, we have an estimate of 46,000 patients, and half of them are found in the AlAhsa district.[2]

We reported two patients diagnosed with COVID-19 since the start of the pandemic among 3565 and four fatalities.

First patient

A 34-year-old male with homozygous SS and history of the left hip replacement (January 2020), and ACS 2 weeks before the pandemic was officially announced. He was diagnosed with COVID-19 by positive polymerase chain reaction (PCR) testing after active surveillance in his village; otherwise, he was completely asymptomatic. He was admitted with neither respiratory symptoms nor fever. He received hydroxychloroquine (HCQ), enoxaparin, and ceftriaxone. Repeated nasopharyngeal swab for SARS-CoV-2 PCR was negative and he was discharged from the hospital [Table 1].
Table 1: Clinical summary of sickle cell disease patients with coronavirus disease 2019

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Second patient

A 40-year-old female with homozygous SS presented with an acute painful crisis, generalized fatigue and she was in close contact with COVID-19 patients. She did not have respiratory symptoms. She was tested positive for COVID-19 by PCR. She had a history of repeated painful crises and was not on regular follow-up. The patient received tramadol, oxygen therapy (HCQ), enoxaparin, and ceftriaxone. Repeated nasopharyngeal swab for SARS-CoV-2 PCR was negative, and she was discharged on the 10th day [Table 1].

There are three factors that make SCD a risk factor for COVID-19 infection. The first is that SCD leads to an immunocompromised state due to splenic dysfunction as early as the 1st year of life and subsequent autosplenectomy or surgical splenectomy. Two mechanisms are affected, trapping the organisms from blood vessels and opsonization which is a prerequisite for defending against Gram-positive as well as Gram-negative encapsulated organisms.[1] The second factor is the prothrombotic state due to the activation of platelets, reduced antifibrinolytic mechanism, and reduction of some clotting factors. Those patients populations have preexisted pulmonary complications caused by ACS.[3] The third factor is pulmonary comorbidities due to SCD complications such as pulmonary hypertension, and pulmonary embolism which is four times higher versus the general population.[3]

Recently, a hypothesis by two scientists indicated that SARS-CoV-2 is attacking the heme group, making the hemoglobin inefficient for gas exchange, and contributes to the inflammatory storm, without strongin vitro orin vivo proof.[4] Based on our population pool and expectations of high numbers of infected persons, we note the following:

  1. SCD is not a risk for viral infection such as SARS-CoV-2, but we should be careful about the certain population with pulmonary complications such as ACS, and pulmonary embolism
  2. The reduction of reported SCD with COVID-19 could be because these patients were kept at home, and away from contact with COVID-19 patients
  3. We should encourage health-care professionals to report their experience of COVID-19 in SCD patients
  4. In many countries, SCD is associated with glucose 6-phosphate dehydrogenase deficiency and for those diagnosed with COVID-19, we need to be vigilant in treating them with HCQ or chloroquine, due to the risk of hemolysis in these anemic patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: A review. Int J Infect Dis 2010;14:e2-12.  Back to cited text no. 1
Alsultan A, Jastaniah W, Al Afghani S, Al Bagshi MH, Nasserullah Z, Al-Suliman AM, et al. Demands and challenges for patients with sickle-cell disease requiring hematopoietic stem cell transplantation in Saudi Arabia. Pediatr Transplant 2016;20:831-5.  Back to cited text no. 2
Stein PD, Beemath A, Meyers FA, Skaf E, Olson RE. Deep venous thrombosis and pulmonary embolism in hospitalized patients with sickle cell disease. Am J Med 2006;119:897.e7-11.  Back to cited text no. 3
Wenzhong L, Hualan L. COVID-19: Attacks the 1-Beta Chain of Hemoglobin and Captures the Porphyrin to Inhibit Human Heme Metabolism; 27 April, 2020. Available from: https://chemrxiv.org/articles/COVID19_Disease_ORF8_and_Surface_Glycoprotein_Inhibit_Heme_Metabolism_by_Binding_to_Porphyrin/11938173. [Last accessed on 2020 Jun 02].  Back to cited text no. 4


  [Table 1]

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