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Best Practice & Research. Clinical... Sep 2022Throughout the Coronavirus Disease 2019 (COVID-19) pandemic, understanding the effects of COVID-19 on persons with Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT)... (Review)
Review
Throughout the Coronavirus Disease 2019 (COVID-19) pandemic, understanding the effects of COVID-19 on persons with Sickle Cell Disease (SCD) and Sickle Cell Trait (SCT) has garnered interest. Patients with SCD diagnosed with COVID-19 utilize the emergency department and are hospitalized at significantly higher rates compared to the general population, with vaso-occlusive crisis and acute chest syndrome as the leading presentations. Whether SCD alone increases the likelihood of severe COVID-19 illness remains uncertain; however, potential risk factors for severe disease among patients with SCD include older age, frequent acute care visits for pain, haemoglobin SC disease, and pre-existing end-organ disease. SCT status may also influence COVID-19 outcomes, particularly among those with pre-existing co-morbidities. Corticosteroids in patients with SCD and COVID-19 should be used with extreme caution given strong associations between corticosteroid exposure and severe vaso-occlusive crisis, with prophylactic transfusion administered if corticosteroids are deemed necessary. Hydroxyurea may be protective in COVID-19.
Topics: Humans; Sickle Cell Trait; COVID-19; Anemia, Sickle Cell; Hydroxyurea; Risk Factors
PubMed: 36494153
DOI: 10.1016/j.beha.2022.101382 -
Blood Reviews May 2023The hematologic disorders myelodysplastic syndromes and beta-thalassemia are characterized by ineffective erythropoiesis and anemia, often managed with regular blood... (Review)
Review
The hematologic disorders myelodysplastic syndromes and beta-thalassemia are characterized by ineffective erythropoiesis and anemia, often managed with regular blood transfusions. Erythropoiesis, the process by which sufficient numbers of functional erythrocytes are produced from hematopoietic stem cells, is highly regulated, and defects can negatively affect the proliferation, differentiation, and survival of erythroid precursors. Treatments that directly target the underlying mechanisms of ineffective erythropoiesis are limited, and management of anemia with regular blood transfusions imposes a significant burden on patients, caregivers, and health care systems. There is therefore a strong unmet need for treatments that can restore effective erythropoiesis. Novel therapies are beginning to address this need by targeting a variety of mechanisms underlying erythropoiesis. Herein, we provide an overview of the role of ineffective erythropoiesis in myelodysplastic syndromes and beta-thalassemia, discuss unmet needs in targeting ineffective erythropoiesis, and describe current management strategies and emerging treatments for these disorders.
Topics: Humans; beta-Thalassemia; Erythropoiesis; Erythrocytes; Hematologic Diseases; Myelodysplastic Syndromes
PubMed: 36577601
DOI: 10.1016/j.blre.2022.101039 -
Blood Jun 2021
Topics: Anemia, Sickle Cell; Erythrocytes; Humans
PubMed: 34081122
DOI: 10.1182/blood.2021011143 -
Clinics (Sao Paulo, Brazil) 2022This work aimed to better understand the impact of pandemics of respiratory viruses on children with hemoglobinopathies through a comprehensive review of the literature.... (Review)
Review
This work aimed to better understand the impact of pandemics of respiratory viruses on children with hemoglobinopathies through a comprehensive review of the literature. MEDLINE, SCIELO, LILACS, and PUBMED were used as data sources to find articles without time period restrictions. Previous observations suggest that patients with hemoglobinopathies are a group especially susceptible to the complications of viral respiratory infections, with greater morbidity and mortality related to them. Within this context, this review found that, during the 2009 H1N1 pandemic, the risk of hospitalization in children and adults increased, especially in patients with a history of complications such as acute chest syndrome. In addition, the Coronavirus Disease 2019 (COVID-19) pandemic appears to have less repercussion among children with hemoglobinopathies compared to adults, similar to what is seen in the general population. In the H1N1 pandemic, patients with hemoglobinopathies behaved as a group more susceptible to complications, with increased morbidity and mortality. However, for COVID-19, the existing data to date on these patients do not show the same clinical impact. Thus, although these children deserve attention in case of infection due to their potential risks, they seem to have a favorable evolution.
Topics: Adult; COVID-19; Child; Hemoglobinopathies; Humans; Influenza A Virus, H1N1 Subtype; Pandemics; SARS-CoV-2
PubMed: 35113785
DOI: 10.1016/j.clinsp.2021.100004 -
Blood Mar 2023
Topics: Humans; beta-Thalassemia; Thalassemia; Mutation
PubMed: 36893006
DOI: 10.1182/blood.2022019350 -
The Lancet. Global Health Jan 2022
Topics: Developing Countries; Hemoglobin, Sickle; Hemoglobinopathies; Humans; Infant, Newborn; Neonatal Screening
PubMed: 34919844
DOI: 10.1016/S2214-109X(21)00559-3 -
British Journal of Haematology Aug 2019
Topics: Adult; Brain; Cognition; Humans; Italy; Thalassemia; beta-Thalassemia
PubMed: 31115039
DOI: 10.1111/bjh.15956 -
Der Ophthalmologe : Zeitschrift Der... Oct 2021Sickle cell disease (SCD) is a hereditary hemoglobinopathy, which leads to microcirculatory disturbances of various organ systems through recurrent vaso-occlusive... (Review)
Review
BACKGROUND
Sickle cell disease (SCD) is a hereditary hemoglobinopathy, which leads to microcirculatory disturbances of various organ systems through recurrent vaso-occlusive episodes, with a possibly fatal outcome. Sickle cell retinopathy (SCR) is the best described ocular manifestation of SCD. Irrespective of the presence of peripheral SCR, sickle cell maculopathy (SCM) can occur early in the course of the disease.
METHODS
Review of the international and German literature on ocular involvement in SCD with a focus on SCR and SCM and an overview of current systemic therapeutic approaches in SCD on the occasion of the presentation of two patients with HbSS SCD.
RESULTS AND CONCLUSION
In contrast to SCR, SCM with temporal thinning of the inner retinal layers has only been increasingly described in the literature in the last 5 years, with the advent of SD-OCT and OCTA. Irrespective of the presence of SCR, as many as about half of the patients may develop SCM early in the course of the disease. As a result of progress in systemic therapeutic options and due to migration, the clinical picture will occur more often also in Germany. By knowing about this complication of SCD an early diagnosis can be made and unnecessary diagnostics can be avoided.
Topics: Anemia, Sickle Cell; Humans; Macular Degeneration; Microcirculation; Retina; Retinal Diseases
PubMed: 33502544
DOI: 10.1007/s00347-020-01319-8 -
American Journal of Hematology Jan 2020
Topics: Humans; beta-Thalassemia; delta-Thalassemia
PubMed: 31444804
DOI: 10.1002/ajh.25623 -
Hematology/oncology Clinics of North... Dec 2022Sickle cell disease (SCD) is a genetic hemoglobinopathy associated with extensive morbidity and early mortality. While there have been recent improvements in available... (Review)
Review
Sickle cell disease (SCD) is a genetic hemoglobinopathy associated with extensive morbidity and early mortality. While there have been recent improvements in available disease-modifying therapies for SCD, cardiopulmonary complications remain a major risk factor for death in this population. We provide an overview of current knowledge regarding several of the major acute and chronic cardiopulmonary complications in SCD, including: acute chest syndrome, airway disease, lung function abnormalities, nocturnal hypoxemia and sleep disordered breathing, pulmonary vascular disease, and sickle cell cardiomyopathy.
Topics: Humans; Anemia, Sickle Cell; Sleep Apnea Syndromes; Vascular Diseases
PubMed: 36400540
DOI: 10.1016/j.hoc.2022.07.014