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Sickle Cell Disease: Current Understanding and Future Options
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Sickle Cell Disease: Current Understanding and Future Options

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Hematology".

Deadline for manuscript submissions: closed (15 December 2022) | Viewed by 10892

Special Issue Editor

Dr. Eleni Gavriilaki

E-Mail Website
Guest Editor
Hematology Department-BMT Unit, G Papanicolaou Hospital, Exochi, 57010 Thessaloniki, Greece
Interests: thrombotic microangiopathy; complement; complement inhibitors; hematopoietic cell transplantation; atypical hemolytic uremic syndrome; COVID-19; thrombotic thrombocytopenic purpura
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Sickle cell disease (SCD) is a widely spread inherited hemoblobinopathy that includes a group of congenital hemolytic anemias, all characterized by the predominance of sickle hemoglobin (HbS). Its features are anemia, predisposal to bacterial infections, and complications (such as vaso-occlusive crisis (VOC) or delayed hemolytic transfusion reaction (DHTR)), which lead to increased rate of morbidity and mortality even in the era of hydroxyurea. The interaction between sickle cells, neutrophils, platelets, or endothelial cells in small vessels results in hemolysis and has been considered the disease’s main pathophysiological mechanism. Complement activation has been reported in small cohorts of SCD patients, but the governing mechanism has not been fully elucidated. This will be important to predict the patient group that would benefit from complement inhibition. Until now, eculizumab-mediated complement inhibition has shown beneficial effects in DHTR, with limited reports in patients with VOC. In the meantime, several innovative agents are under clinical development or have been already introduced in clinical practice. Furthermore, the improvement of long-term complications and quality of life remains under investigation. Therefore, this Special Issue aims to delineate both our current understanding and future options in SCD.

Dr. Eleni Gavriilaki
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • sickle cell disease
  • vaso-occlussive crisis
  • delayed hemolytic transfusion reaction
  • voxelotor
  • hematopoietic cell transplantation
  • hydroxyurea
  • transfusion
  • anemia
  • quality of life

Published Papers (6 papers)

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Editorial

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2 pages, 163 KiB  
Editorial
Sickle Cell Disease: Current Understanding and Future Options
by Christos Varelas and Eleni Gavriilaki
J. Clin. Med. 2023, 12(18), 5943; https://doi.org/10.3390/jcm12185943 - 13 Sep 2023
Viewed by 582
Abstract
Sickle cell disease (SCD) is a prevalent inherited hemoglobin disorder encompassing a cluster of congenital hemolytic anemias, each distinguished by the prevalence of sickle hemoglobin (HbS) [...] Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)

Research

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11 pages, 281 KiB  
Article
COVID-19 and Sickle Cell Disease in the Province of Quebec, Canada: Outcomes after Two Years of the Pandemic
by Mathias Castonguay, Nawar Dakhallah, Justin Desroches, Marie-Laure Colaiacovo, Camille Jimenez-Cortes, Anne-Marie Claveau, Samuel Bérubé, Amer Yassine Hafsaoui, Amalia Souza, Pauline Tibout, Christophe Ah-Yan, Anne-Marie Vincent, Veronique Naessens, Josée Brossard, Sharon Abish, Raoul Santiago, Denis Soulières, Vincent Laroche, Yves Pastore, Thai Hoa Tran and Stéphanie Fortéadd Show full author list remove Hide full author list
J. Clin. Med. 2022, 11(24), 7361; https://doi.org/10.3390/jcm11247361 - 12 Dec 2022
Cited by 6 | Viewed by 1942
Abstract
Background: Patients with sickle cell disease (SCD) are considered at higher risk of severe COVID-19 infection. However, morbidity and mortality rates are variable among countries. To date, there are no published reports that document outcomes of SCD patients with COVID-19 in Canada. Methods: [...] Read more.
Background: Patients with sickle cell disease (SCD) are considered at higher risk of severe COVID-19 infection. However, morbidity and mortality rates are variable among countries. To date, there are no published reports that document outcomes of SCD patients with COVID-19 in Canada. Methods: A web-based registry was implemented in June 2020 capturing outcomes of SCD patients with COVID-19 from March 2020 to April 2022 and comparing them to the general population of Quebec, Canada. Results: After 24 months of the pandemic, 185 SCD patients with confirmed SARS-CoV-2 infection were included in the registry. Overall, the population was young (median age 12 years old) and had few comorbidities. No deaths were reported. Risk of hospitalization and admission to intensive care unit (ICU) because of COVID-19 was higher in patients with SCD than in the general population (relative risks (RR) 5.15 (95% confidence interval (95% CI) 3.84–6.91), p ˂ 0.001 and 4.56 (95% CI 2.09–9.93) p ˂ 0.001). A history of arterial hypertension or acute chest syndrome in the past 12 months was associated with a higher risk of severe disease (RR = 3.06 (95% CI 1.85–5.06) p = 0.008 and 2.27 (95% CI 1.35–3.83) p = 0.01). Hospitalized patients had lower hemoglobin F than non-hospitalized patients (12% vs. 17%, p = 0.02). For those who had access to vaccination at the time of infection, 25 out of 26 patients were adequately vaccinated and had mild disease. Conclusions: The SCD population is at higher risk of severe disease than the general population. However, we report favorable outcomes as no deaths occurred. Registries will continue to be critical to document the impact of novel COVID-19 specific therapy and vaccines for the SCD population. Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)
6 pages, 603 KiB  
Article
Rate of Dental Extractions in Patients with Sickle Cell Disease
by Adeel Ahmad, Diana Mihalca, Ben Stacey, Sayna Samaee, Dipal Mehta, Stephen Hibbs, Tanya Freeman, Basabi Chatterjee, Enamul Ali, Leo Cheng and Dimitris A. Tsitsikas
J. Clin. Med. 2022, 11(20), 6174; https://doi.org/10.3390/jcm11206174 - 19 Oct 2022
Cited by 2 | Viewed by 1275
Abstract
Background: Sickle cell disease is an inherited disorder associated with chronic haemolysis and anaemia, recurrent episodes of pain and potentially multisystem end-organ damage. A lot less is known about the dental health of these patients. Aims: To explore the incidence of severe dental [...] Read more.
Background: Sickle cell disease is an inherited disorder associated with chronic haemolysis and anaemia, recurrent episodes of pain and potentially multisystem end-organ damage. A lot less is known about the dental health of these patients. Aims: To explore the incidence of severe dental disease leading to dental extraction in our sickle cell population. Patient/methods: We undertook an audit looking at the rate of dental extractions, as a composite marker of severe dental disease, among sickle cell patients over a 3-month period. The patients were unselected and approached during routine assessments. We analysed both clinical and laboratory data to look for possible associations between dental disease and sickle cell characteristics. Results: 177 patients were interviewed between February 2022 and April 2022. Overall, 71% of the patients had at least one dental extraction with a median number of teeth extracted of three and a median age at first extraction of 26. More than half of the patients stated that they do not have regular dental check-ups. There were no significant associations with the severity of sickle cell phenotype, baseline Hb or markers of haemolysis. Conclusion: A large number of patients with sickle cell disease require dental extractions at a relatively young age. The lack of any correlation with disease severity suggests that poor engagement with dental services and the underestimation of the importance of dental health are the main factors behind the increased prevalence of severe dental disease. Actively enquiring about dental problems should be part of any routine consultation with these patients, both in primary and specialist care. Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)
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Review

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13 pages, 779 KiB  
Review
Mitochondria: Emerging Consequential in Sickle Cell Disease
by Mohammad S. Akhter, Hassan A. Hamali, Hina Rashid, Gasim Dobie, Aymen M. Madkhali, Abdullah A. Mobarki, Johannes Oldenburg and Arijit Biswas
J. Clin. Med. 2023, 12(3), 765; https://doi.org/10.3390/jcm12030765 - 18 Jan 2023
Cited by 2 | Viewed by 2079
Abstract
Advanced mitochondrial multi-omics indicate a multi-facet involvement of mitochondria in the physiology of the cell, changing the perception of mitochondria from being just the energy-generating organelles to organelles that highly influence cell structure, function, signaling, and cell fate. This sets mitochondrial dysfunction in [...] Read more.
Advanced mitochondrial multi-omics indicate a multi-facet involvement of mitochondria in the physiology of the cell, changing the perception of mitochondria from being just the energy-generating organelles to organelles that highly influence cell structure, function, signaling, and cell fate. This sets mitochondrial dysfunction in the centerstage of numerous acquired and genetic diseases. Sickle cell disease is also being increasingly associated with mitochondrial anomalies and the pathophysiology of sickle cell disease finds mitochondria at crucial intersections in the pathological cascade. Altered mitophagy, increased ROS, and mitochondrial DNA all contribute to the condition and its severity. Such mitochondrial aberrations lead to consequent mitochondrial retention in red blood cells in sickle cell diseases, increased oxidation in the cellular environment, inflammation, worsened vaso-occlusive crisis, etc. There are increasing studies indicating mitochondrial significance in sickle cell disease, consequently providing an opportunity to target it for improving the outcomes of treatment. Identification of the impaired mitochondrial attributes in sickle cell disease and their modulation by therapeutic interventions can impart a better management of the disease. This review aims to describe the mitochondria in the perspective of sicke cell disease so as to provide the reader an overview of the emerging mitochondrial stance in sickle cell disease. Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)
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Other

10 pages, 816 KiB  
Brief Report
Thromboprophylaxis Reduced Venous Thromboembolism in Sickle Cell Patients with Central Venous Access Devices: A Retrospective Cohort Study
by Stéphanie Forté, Gonzalo De Luna, Jameel Abdulrehman, Nafanta Fadiga, Olivia Pestrin, Anne-Laure Pham Hung d’Alexandry d’Orengiani, John Chinawaeze Aneke, Henri Guillet, Dalton Budhram, Anoosha Habibi, Richard Ward, Pablo Bartolucci and Kevin H. M. Kuo
J. Clin. Med. 2022, 11(5), 1193; https://doi.org/10.3390/jcm11051193 - 23 Feb 2022
Cited by 5 | Viewed by 2630
Abstract
Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is [...] Read more.
Sickle cell disease (SCD) induces a chronic prothrombotic state. Central venous access devices (CVADs) are commonly used for chronic transfusions and iron chelation in this population. CVADs are an additional venous thromboembolism (VTE) risk factor. The role of thromboprophylaxis in this setting is uncertain. The objectives are: (1) to determine whether thromboprophylaxis reduces VTE risk in SCD patients with CVAD and (2) to explore characteristics associated with VTE risk. We identified adults with SCD and CVAD intended for chronic use (≥3 months) at two comprehensive SCD centers. Thromboprophylaxis presence; type; intensity; and patient-, catheter-, and treatment-related VTE risk factors were recorded. Among 949 patients, 49 had a CVAD (25 without and 24 with VTE prophylaxis). Thromboprophylaxis type and intensity varied widely. Patients without thromboprophylaxis had higher VTE rates (rate ratio (RR) = 4.0 (95% confidence interval: 1.2–12.6), p = 0.02). Hydroxyurea was associated with lower VTE rates (RR = 20.5 (6.4–65.3), p < 0.001). PICC lines and Vortex and Xcela Power implantable devices were associated with higher rates compared with Port-a-Cath (RR = 5.8 (1.3–25.9), p = 0.02, and RR = 58.2 (15.0–225.0), p < 0.001, respectively). Thromboprophylaxis, hydroxyurea, and CVAD subtype were independently associated with VTE. The potentially protective role of thromboprophylaxis and hydroxyurea for VTE prevention in patients with SCD and CVAD merits further exploration. Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)
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8 pages, 747 KiB  
Brief Report
Immune Response of Adult Sickle Cell Disease Patients after COVID-19 Vaccination: The Experience of a Greek Center
by Christos Varelas, Eleni Gavriilaki, Ioanna Sakellari, Philippos Klonizakis, Evaggelia-Evdoxia Koravou, Ioanna Christodoulou, Ioulia Mavrikou, Andreas Kourelis, Fani Chatzopoulou, Dimitrios Chatzidimitriou, Tasoula Touloumenidou, Apostolia Papalexandri, Achilles Anagnostopoulos and Efthimia Vlachaki
J. Clin. Med. 2022, 11(4), 937; https://doi.org/10.3390/jcm11040937 - 11 Feb 2022
Cited by 6 | Viewed by 1621
Abstract
Vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are essential weapons to control the spread of the coronavirus disease-19 (COVID-19) pandemic and protect immunocompromised patients. With a greater susceptibility to infection, sickle cell disease (SCD) patients are considered as “high risk” patients [...] Read more.
Vaccines against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) are essential weapons to control the spread of the coronavirus disease-19 (COVID-19) pandemic and protect immunocompromised patients. With a greater susceptibility to infection, sickle cell disease (SCD) patients are considered as “high risk” patients during the current COVID-19 pandemic. In our study, we try to determine the immune response of adult SCD patients monitored at our center after the first and second dose of the qualified mRNA vaccines available and correlate them to several disease-specific markers, as well as complement activation. The results demonstrate that the levels of neutralizing antibodies (nAbs) against SARS-CoV-2 were adequate for most patients studied after the second dose and there seemed to be a certain association with complement activation. Further studies are critical to determine the durability of this immune response and the potential benefit of a third dose. Full article
(This article belongs to the Special Issue Sickle Cell Disease: Current Understanding and Future Options)
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