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1.
Mediterr J Hematol Infect Dis ; 12(1): e2020046, 2020.
Article in English | MEDLINE | ID: covidwho-1792270

ABSTRACT

OBJECTIVES: This study aims to investigate, retrospectively, the epidemiological and clinical characteristics, laboratory results, radiologic findings, and outcomes of COVID-19 in patients with transfusion-dependent ß thalassemia major (TM), ß-thalassemia intermedia (TI) and sickle cell disease (SCD). DESIGN: A total of 17 Centers, from 10 countries, following 9,499 patients with hemoglobinopathies, participated in the survey. MAIN OUTCOME DATA: Clinical, laboratory, and radiologic findings and outcomes of patients with COVID-19 were collected from medical records and summarized. RESULTS: A total of 13 patients, 7 with TM, 3 with TI, and 3 with SCD, with confirmed COVID-19, were identified in 6 Centers from different countries. The overall mean age of patients was 33.7±12.3 years (range:13-66); 9/13 (69.2%) patients were females. Six patients had pneumonia, and 4 needed oxygen therapy. Increased C-reactive protein (6/10), high serum lactate dehydrogenase (LDH; 6/10), and erythrocyte sedimentation rate (ESR; 6/10) were the most common laboratory findings. 6/10 patients had an exacerbation of anemia (2 with SCD). In the majority of patients, the course of COVID-19 was moderate (6/10) and severe in 3/10 patients. A 30-year-old female with TM, developed a critical SARS-CoV-2 infection, followed by death in an Intensive Care Unit. In one Center (Oman), the majority of suspected cases were observed in patients with SCD between the age of 21 and 40 years. A rapid clinical improvement of tachypnea/dyspnea and oxygen saturation was observed, after red blood cell exchange transfusion, in a young girl with SCD and worsening of anemia (Hb level from 9.2 g/dl to 6.1g/dl). CONCLUSIONS: The data presented in this survey permit an early assessment of the clinical characteristics of COVID 19 in different countries. 70% of symptomatic patients with COVID- 19 required hospitalization. The presence of associated co-morbidities can aggravate the severity of COVID- 19, leading to a poorer prognosis irrespective of age.

2.
Hemoglobin ; 45(6): 371-379, 2021 Nov.
Article in English | MEDLINE | ID: covidwho-1240824

ABSTRACT

During the coronavirus-19 disease (COVID-19) pandemic, several studies were performed to determine the mortality and incidence rates of coronavirus infection among patients with hemoglobinopathies. However, there has been no systematic approach or meta-analysis to evaluate the results worldwide. This meta-analysis summarized the existing evidence of incidence and mortality rates of COVID-19 and related risk factors among patients with hemoglobinopathies with a focus on ß-thalassemia (ß-thal) and sickle cell disease. The review was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses checklist. Two authors independently screened the articles, extracted eligible ones, and assessed the quality of studies using the Joanna Briggs Institute (JBI) checklist. The collected data were analyzed by the Stata software. The amount of heterogeneity was demonstrated by the I2 test. The incidence of COVID-19 among patients with a hemoglobinopathy, ß-thal and sickle cell disease was 4.44, 1.34, and 17.22 per 100,000 person-day, respectively, to June 15 2020. The mortality rate of COVID-19 in patients with hemoglobin (Hb) disorders was calculated as 1.07 per 1000 person-day in the same period. Our findings showed a higher incidence rate of COVID-19 in sickle cell disease patients compared to the general population. A slightly higher mortality rate was also observed in patients with hemoglobinopathies compared to the general population, possibly due to the associated risk factors and comorbidities in this vulnerable group, which underscore special care, timely diagnosis and management along with current immunization, were crucial in decreasing the frequency, disease severity and mortality of these patients.


Subject(s)
Anemia, Sickle Cell , COVID-19 , Hemoglobinopathies , beta-Thalassemia , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , COVID-19/epidemiology , Hemoglobinopathies/epidemiology , Humans , Incidence , beta-Thalassemia/epidemiology
3.
Case Rep Crit Care ; 2021: 6617362, 2021.
Article in English | MEDLINE | ID: covidwho-1208756

ABSTRACT

As the pandemic continues to evolve, more cases of COVID-19 in pediatric patients are being detected. A 12-year-old boy with HbSC disease alpha-thalassemia trait presented to a pediatric emergency room with fever and weakness. His vital signs were notable for fever, tachypnea, and tachycardia. His physical exam was concerning for increased work of breathing. He tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) by PCR although his hemoglobin level remained near his baseline. His chest radiograph showed a retrocardiac opacity concerning for evolving acute chest syndrome. He decompensated quickly requiring invasive mechanical ventilation and exchange transfusion. He received hydroxychloroquine, broad-spectrum antibiotics, and enoxaparin for DVT prophylaxis. Despite showing clinical signs of improvement, he became acutely hypoxemic and suffered a cardiac arrest. We believe this to be an unusual case of a pediatric patient with HbSC disease and COVID-19. We outline clearly the course of illness and treatments trialed, which can prove beneficial to providers facing similar challenges as this virus continues to strike areas around the world. Although children have significantly better outcomes than adults, providers must remain vigilant while treating any patient with a hemoglobinopathy in the setting of severe COVID-19.

4.
Orphanet J Rare Dis ; 16(1): 114, 2021 03 01.
Article in English | MEDLINE | ID: covidwho-1112440

ABSTRACT

BACKGROUND: Hydroxyurea is one of the earliest drugs that showed promise in the management of haemoglobinopathies that include ß-thalassaemia and sickle cell disease. Despite this, many aspects of hydroxyurea are either unknown or understudied; specifically, its usefulness in ß-thalassaemia major and haemoglobin E ß-thalassaemia is unclear. However, during COVID-19 pandemic, it has become a valuable adjunct to transfusion therapy in patients with ß-haemoglobinopathies. In this review, we aim to explore the available in vitro and in vivo mechanistic data and the clinical utility of hydroxyurea in ß-haemoglobinopathies with a special emphasis on its usefulness during the COVID-19 pandemic. MAIN BODY: Hydroxyurea is an S-phase-specific drug that reversibly inhibits ribonucleoside diphosphate reductase enzyme which catalyses an essential step in the DNA biosynthesis. In human erythroid cells, it induces the expression of γ-globin, a fetal globin gene that is suppressed after birth. Through several molecular pathways described in this review, hydroxyurea exerts many favourable effects on the haemoglobin content, red blood cell indices, ineffective erythropoiesis, and blood rheology in patients with ß-haemoglobinopathies. Currently, it is recommended for sickle cell disease and non-transfusion dependent ß-thalassaemia. A number of clinical trials are ongoing to evaluate its usefulness in transfusion dependent ß-thalassaemia. During the COVID-19 pandemic, it was widely used as an adjunct to transfusion therapy due to limitations in the availability of blood and logistical disturbances. Thus, it has become clear that hydroxyurea could play a remarkable role in reducing transfusion requirements of patients with haemoglobinopathies, especially when donor blood is a limited resource. CONCLUSION: Hydroxyurea is a well-tolerated oral drug which has been in use for many decades. Through its actions of reversible inhibition of ribonucleoside diphosphate reductase enzyme and fetal haemoglobin induction, it exerts many favourable effects on patients with ß-haemoglobinopathies. It is currently approved for the treatment of sickle cell disease and non-transfusion dependent ß-thalassaemia. Also, there are various observations to suggest that hydroxyurea is an important adjunct in the treatment of transfusion dependent ß-thalassaemia which should be confirmed by randomised clinical trials.


Subject(s)
COVID-19/drug therapy , Hemoglobinopathies/drug therapy , Hydroxyurea/therapeutic use , Bloodless Medical and Surgical Procedures , Enzyme Inhibitors/therapeutic use , Humans , Ribonucleoside Diphosphate Reductase/antagonists & inhibitors
5.
Transfus Apher Sci ; 60(3): 103080, 2021 Jun.
Article in English | MEDLINE | ID: covidwho-1078214

ABSTRACT

The world has been encountered with COVID-19 pandemic since at the beginning of 2020 and the number of infected people by COVID-19 is increasing every day. Despite various studies conducted by researchers and doctors, no treatment has been developed until now, therefore self-protection and isolation are strongly recommended to stop the spread of the virus. The elderly population and people with chronic diseases such as hypertension, cardiovascular diseases, diabetes, and cancer are categorized as risk groups, however, we suggest that people with hemoglobinopathies or porphyria can be described as risk groups as well. Current in silico studies have revealed that the COVID-19 virus can attack heme and hemoglobin metabolisms which are responsible for the oxygen transport to the tissues, iron metabolism, elevated levels of oxidative stress, and tissue damage. Data of the in silico study have been supported with the biochemistry and hemogram results of the COVID-19 patients, for instance hemoglobin levels decreased and serum ferritin and C-reactive protein levels increased. Indicated biochemistry biomarkers are tightly associated with inflammation, iron overload, and oxidative stress. In conclusion, since people with hemoglobinopathies or porphyria have already impaired heme and hemoglobin metabolism, COVID-19 infection can enhance the adverse effects of impaired hemoglobin metabolism and accelerate the progression of severe symptoms in patients with hemoglobinopathies or porphyria compared to the normal individuals. Thus those people can be considered as a risk group and extra precautions should be applied for them to protect them.


Subject(s)
COVID-19/blood , COVID-19/epidemiology , Hematologic Diseases/epidemiology , Oxidative Stress/genetics , SARS-CoV-2/pathogenicity , COVID-19/virology , Hematologic Diseases/virology , Humans , Pandemics
6.
Clin Case Rep ; 9(2): 861-865, 2021 Feb.
Article in English | MEDLINE | ID: covidwho-985980

ABSTRACT

To optimize care for patients with hemoglobinopathies, frequent screening for COVID-19 is prudent as viral kinetics in asplenic patients are unknown and differentiating prolonged viral shedding versus reinfection remains a challenge.

7.
Hematol Transfus Cell Ther ; 43(1): 87-100, 2021.
Article in English | MEDLINE | ID: covidwho-956039

ABSTRACT

INTRODUCTION: It is important to know if patients with hemoglobinopathy could be more susceptible to COVID-19. OBJECTIVE: Analyze SARS-CoV-2 infection in pediatric patients with hemoglobinopathy. METHODS: Using the online platforms LILACS, PUBMED and EMBASE, on 17- JUL-2020 a search was made for the terms COVID-19 and SARS-CoV-2 associated with "sickle cell", "thalassemia" and "hemoglobinopathy". RESULTS: There were 623 pediatric and adult patients with sickle cell disease (SCD) or beta thalassemia (BT) and COVID-19. Total mortality rate was 6.42%. No pediatric patient with BT has been described. So, our analysis focused on children and adolescents with SCD: there were 121 pediatric patients, one adolescent died, prophylactic anticoagulation was prescribed to six patients, 11.76% needed intensive care unit, blood transfusion was prescribed in 29.70%. Vaso-occlusive crisis (VOC) and acute chest syndrome (ACS) were the main clinical manifestations in SCD. DISCUSSION: Pediatric patients with SCD and COVID-19 have a low mortality rate when compared to adults, although is higher than the global pediatric population with COVID-19 (0-0.67%). The comorbidities associated with age and the long-term complications inherent to hemoglobinopathies may contribute to the increased mortality outside the pediatric age group. In SCD the clinical manifestations, both in children and adults, are VOC and ACS, and there was increase in blood requirement. Pediatric SCD patients with COVID-19 need more intensive care unit than the global pediatric population (3.30%). CONCLUSION: Despite pediatric population with SCD needs more intensive care, the outcome after infection by COVID-19 is favorable.

8.
Front Med (Lausanne) ; 7: 306, 2020.
Article in English | MEDLINE | ID: covidwho-615502

ABSTRACT

The coronavirus disease 2019 (COVID-19) is an emerging infectious disease that has become a global public health concern after being first reported in China and has subsequently spread worldwide. It causes mild to severe respiratory illness with some flu-like symptoms. The causal virus behind this disease, SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2), conceivably attacks the receptors of the respiratory system of the human body but has no strict evidence of attacking the blood cells yet. However, patients with hemoglobin disorders (e.g., sickle cell anemia, thalassemia) are vulnerable to this global health situation due to their clinical complications. Such patients are generally more prone to viral and bacterial infections, which can worsen their physical condition. Some of these patients present immunocompromised conditions, e.g., splenectomized or post-transplant patients. Therefore, they should follow some preventive steps such as shielding as well as the general guidelines for the COVID-19 pandemic. Transfusion dependent patients require regular monitoring for iron overload, and iron chelation therapy may be stopped by the physician depending on the situation. This article reviews the management strategies and provides some crucial recommendations for people in the corner with hemoglobin disorders.

9.
Eur J Haematol ; 105(4): 378-386, 2020 Oct.
Article in English | MEDLINE | ID: covidwho-612275

ABSTRACT

OBJECTIVES: Many patients with haemoglobinopathies, including thalassaemia and sickle cell disease, are at increased risk of developing severe complications from the coronavirus disease 2019 (COVID-19). Although epidemiologic evidence concerning the novel coronavirus (SARS-CoV-2) infection in these patients is currently lacking, the COVID-19 pandemic represents a significant challenge for haemoglobinopathy patients, their families and their attending physicians. METHODS: The present statement summarizes the key challenges concerning the management of haemoglobinopathies, with particular focus on patients with either transfusion-dependent or non-transfusion-dependent thalassaemia, identifies the gaps in knowledge and suggests measures and strategies to deal with the pandemic, based on available evidence and expert opinions. Key areas covered include patients' risk level, adaptation of haemoglobinopathy care, safety of blood transfusions, blood supply challenges, and lifestyle and nutritional considerations. CONCLUSIONS: The proposed measures and strategies may be useful as a blueprint for other disorders which require regular hospital visits, as well as for the timely adaptation of patient care during similar future pandemics.


Subject(s)
COVID-19/complications , Thalassemia/complications , Algorithms , Blood Transfusion , COVID-19/prevention & control , COVID-19/transmission , COVID-19 Testing , Health Personnel , Humans , International Agencies , Pandemics , Patient Care , Risk Factors , SARS-CoV-2 , Safety , Thalassemia/therapy
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