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1.
Indian Pediatr ; 59(7): 531-534, 2022 Jul 15.
Article in English | MEDLINE | ID: covidwho-1958369

ABSTRACT

OBJECTIVE: To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks). METHODS: This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO. RESULTS: 50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01). CONCLUSIONS: Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.


Subject(s)
COVID-19 , Thalassemia , beta-Thalassemia , COVID-19/epidemiology , Child , Ferritins , Humans , Lung/diagnostic imaging , Pandemics , SARS-CoV-2
2.
Indian Pediatr ; 59(7): 531-534, 2022 Jul 15.
Article in English | MEDLINE | ID: covidwho-1857705

ABSTRACT

OBJECTIVE: To evaluate pulmonary functions in children with transfusion-dependent thalassemia, and its reversal (lung dysfunction) using intensive intravenous chelation with desferrioxamine (DFO) (4 weeks). METHODS: This descriptive study enrolled 77 children with transfusion-dependent thalassemia. Pulmonary function test (PFT) and iron load (serum ferritin (SF) and T2* MRI of heart and liver) were done. PFT included spirometry, total lung capacity (TLC) by helium dilution test and diffusion capacity by carbon monoxide (DLCO). Follow-up PFT was available for 13 children with moderate to severe lung dysfunction given intravenous DFO. RESULTS: 50 (68.8%) patients had lung dysfunction, most commonly diffusional impairment (48; 96%), and reduced TLC (11; 22%); and none had obstructive pattern. 9 (81.8%) patients with restrictive defect had moderate to severely deranged DLCO. PFT and T2* MRI values were inversely correlated with serum ferritin. Among 13 patients receiving intensive chelation for 4 weeks, significant improvement was noticed in forced expiratory volume in one minute/ forced vital capacity ratio (DFEV1/FVC) (P=0.009), DDLCO (P=0.006) and DSF (P=0.01). CONCLUSIONS: Pulmonary dysfunction is common in children with multi-transfused thalassemia, and routine screening by PFT needs to be part of the management guidelines.


Subject(s)
COVID-19 , Thalassemia , beta-Thalassemia , COVID-19/epidemiology , Child , Ferritins , Humans , Lung/diagnostic imaging , Pandemics , SARS-CoV-2
4.
J Cell Mol Med ; 26(9): 2520-2528, 2022 05.
Article in English | MEDLINE | ID: covidwho-1769729

ABSTRACT

Although numerous patient-specific co-factors have been shown to be associated with worse outcomes in COVID-19, the prognostic value of thalassaemic syndromes in COVID-19 patients remains poorly understood. We studied the outcomes of 137 COVID-19 patients with a history of transfusion-dependent thalassaemia (TDT) and transfusion independent thalassaemia (TIT) extracted from a large international cohort and compared them with the outcomes from a matched cohort of COVID-19 patients with no history of thalassaemia. The mean age of thalassaemia patients included in our study was 41 ± 16 years (48.9% male). Almost 81% of these patients suffered from TDT requiring blood transfusions on a regular basis. 38.7% of patients were blood group O. Cardiac iron overload was documented in 6.8% of study patients, whereas liver iron overload was documented in 35% of study patients. 40% of thalassaemia patients had a history of splenectomy. 27.7% of study patients required hospitalization due to COVID-19 infection. Amongst the hospitalized patients, one patient died (0.7%) and one patient required intubation. Continuous positive airway pressure (CPAP) was required in almost 5% of study patients. After adjustment for age-, sex- and other known risk factors (cardiac disease, kidney disease and pulmonary disease), the rate of in-hospital complications (supplemental oxygen use, admission to an intensive care unit for CPAP therapy or intubation) and all-cause mortality was significantly lower in the thalassaemia group compared to the matched cohort with no history of thalassaemia. Amongst thalassaemia patients in general, the TIT group exhibited a higher rate of hospitalization compared to the TDT group (p = 0.001). In addition, the rate of complications such as acute kidney injury and need for supplemental oxygen was significantly higher in the TIT group compared to the TDT group. In the multivariable logistic regression analysis, age and history of heart or kidney disease were all found to be independent risk factors for increased in-hospital, all-cause mortality, whereas the presence of thalassaemia (either TDT or TIT) was found to be independently associated with reduced all-cause mortality. The presence of thalassaemia in COVID-19 patients was independently associated with lower in-hospital, all-cause mortality and few in-hospital complications in our study. The pathophysiology of this is unclear and needs to be studied in vitro and in animal models.


Subject(s)
COVID-19 , Iron Overload , Thalassemia , COVID-19/complications , Female , Hospitals , Humans , Iron Overload/etiology , Male , Oxygen , Registries , Thalassemia/complications , Thalassemia/therapy
5.
Br J Haematol ; 197(5): 576-579, 2022 06.
Article in English | MEDLINE | ID: covidwho-1741343

ABSTRACT

Patients with transfusion-dependent thalassaemia (TDT) are considered an at increased-risk population for severe and/or morbid coronavirus disease 2019 (COVID-19) infection. Timely vaccination is the main preventive method for severe COVID-19. Different adverse events and reactions after vaccination have been reported, with severe ones being extremely rare. Patients with TDT may have altered immunity due to chronic transfusions, iron overload and chelation therapy, and splenic dysfunction. Here, we show that adult patients with TDT following vaccination with the novel messenger RNA vaccines have mild adverse events and can produce protective antibodies comparable to the healthy population.


Subject(s)
COVID-19 , Thalassemia , Adult , Antibodies, Viral , COVID-19/prevention & control , Humans , Immunity , SARS-CoV-2 , Thalassemia/complications , Thalassemia/therapy , Vaccination/adverse effects
6.
Am J Hematol ; 97(2): E75-E78, 2022 02 01.
Article in English | MEDLINE | ID: covidwho-1565162
7.
J Hosp Infect ; 115: 51-58, 2021 Sep.
Article in English | MEDLINE | ID: covidwho-1379144

ABSTRACT

BACKGROUND: Occurrence of hepatitis C virus (HCV) infection is reduced by effective risk management procedures, but patient-to-patient transmission continues to be reported in healthcare settings. AIM: To report the use of phylogenetic analysis in the clinical risk management of an HCV outbreak among 128 thalassaemia outpatients followed at a thalassaemia centre of an Italian hospital. METHODS: Epidemiological investigation and root-cause analysis were performed. All patients with acute hepatitis and known chronic infection were tested for HCV RNA, HCV genotyping, and NS3, NS5A, and NS5B HCV genomic region sequencing. To identify transmission clusters, phylogenetic trees were built for each gene employing Bayesian methods. FINDINGS: All patients with acute hepatitis were infected with HCV genotype 1b. Root-cause analysis, including a lookback procedure, excluded blood donors as the source of HCV transmission. The phylogenetic analysis, conducted on seven patients with acute infection and eight patients with chronic infection, highlighted four transmission clusters including at least one patient with chronic and one patient with acute HCV infection. All patients in the same cluster received a blood transfusion during the same day. Two patients with acute hepatitis spontaneously cleared HCV within four weeks and nine patients received ledipasvir plus sofosbuvir for six weeks, all achieving a sustained virological response. CONCLUSION: Combined use of root-cause analysis and molecular epidemiology was effective in ascertaining the origin of the HCV outbreak. Antiviral therapy avoided the chronic progression of the infection and further spread in care units and in the family environment.


Subject(s)
Hepatitis C , Thalassemia , Antiviral Agents/therapeutic use , Bayes Theorem , Disease Outbreaks , Genotype , Hepacivirus/genetics , Hepatitis C/epidemiology , Humans , Italy/epidemiology , Phylogeny , Risk Management , Thalassemia/complications , Thalassemia/epidemiology , Thalassemia/therapy
8.
Transfus Clin Biol ; 29(1): 70-74, 2022 Feb.
Article in English | MEDLINE | ID: covidwho-1317783

ABSTRACT

INTRODUCTION: Because of iron overload complications, thrombosis and infectious predisposition, patients with severe forms of thalassemia are likely to be at increased risk of COVID-19 complications. RESULTS: A national survey conducted during the year 2020 across the French reference centers for hemoglobinopathies identified 16 cases of COVID-19 confirmed by RT-PCR in beta-thalassemia patients. Their age ranged from 11 months to 60 years. 15 patients were transfusion-dependent and 6 were splenectomized. Concerning iron overload related complications, none had diabetes or cirrhosis and only one had experienced heart failure. All 4 pediatric patients were pauci-symptomatic during the viral episode. Three patients (41, 49 and 57 years old) developed COVID-19 pneumonia requiring oxygen therapy without the need for mechanical ventilation. Neutropenia (absolute neutrophils count <0.5 10 9/L) was observed in 2 patients receiving long-term treatment with hydroxycarbamide and deferiprone. No thrombosis event, organ failure or death occurred. All patients recovered. CONCLUSION: Severity of COVID-19 in this population of young and middle-aged patients appeared increased compared to the general population but remained mild to moderate as already described in the few series reported in the literature. Occurrence of adverse events related to chronic treatment administered in thalassemia disease may be favored by the infectious episode.


Subject(s)
COVID-19 , Iron Overload , Thalassemia , beta-Thalassemia , Child , Humans , Infant , Iron Overload/epidemiology , Iron Overload/etiology , Middle Aged , SARS-CoV-2 , beta-Thalassemia/complications , beta-Thalassemia/therapy
14.
Transfus Clin Biol ; 28(2): 129-131, 2021 05.
Article in English | MEDLINE | ID: covidwho-1189034
15.
Pan Afr Med J ; 38: 33, 2021.
Article in English | MEDLINE | ID: covidwho-1134472

ABSTRACT

The COVID-19 pandemic could have a major impact on the capacity of health systems to continue the delivery of essential health service. While health systems around the world are being challenged by increasing demand for care of COVID-19 patients, it is critical to maintain preventive and curative services, especially for the most vulnerable populations such people living with chronic conditions like thallasemics. In this context and since the start of the SARS-CoV-2 health crisis, the National Blood Transfusion Center of Morocco has ranked among its priorities the need to maintain transfusion management for chronic polytransfused patients, particularly those with thalassemia. We report in this paper, the case of a thallasemic patient whose transfusion management was disrupted by the restrictive measures introduced by the Moroccan authorities and for which the National Blood Center of Morocco provided effective support.


Subject(s)
Blood Transfusion , COVID-19 , Continuity of Patient Care , Thalassemia/therapy , Adolescent , Female , Humans , Morocco
17.
Med Sci Monit Basic Res ; 27: e929207, 2021 Jan 05.
Article in English | MEDLINE | ID: covidwho-1006822

ABSTRACT

As of November 25, 2020, over 60 million people have been infected worldwide by COVID-19, causing almost 1.43 million deaths. Puzzling low incidence numbers and milder, non-fatal disease have been observed in Thailand and its Southeast (SE) Asian neighbors. Elusive genetic mechanisms might be operative, as a multitude of genetic factors are widely shared between the SE Asian populations, such as the more than 60 different thalassemia syndromes (principally dominated by the HbE trait). In this study, we have plotted COVID-19 infection and death rates in SE Asian (SEA) countries against heterozygote HbE and thalassemia carrier prevalence. COVID-19 infection and death incidence numbers appear inversely correlated with the prevalence of HbE and thalassemia heterozygote populations. We posit that the evolutionary protective effect of the HbE and other thalassemic variants against malaria and the dengue virus may extend its advantage to resistance to COVID-19 infection, as HbE heterozygote population prevalence appears to be positively correlated with immunity to COVID-19. Host immune system modulations induce antiviral interferon responses and alter structural protein integrity, thereby inhibiting cellular access and viral replication. These changes are possibly engendered by HbE carrier miRNAs. Proving this hypothesis is important, as it may shed light on the mechanism of viral resistance and lead to novel antiviral treatments. This development can thus guide decision-making and action to prevent COVID-19 infection.


Subject(s)
COVID-19/genetics , COVID-19/prevention & control , Disease Resistance/genetics , Disease Susceptibility , Hemoglobin E/genetics , Antiviral Agents/therapeutic use , COVID-19/epidemiology , COVID-19/immunology , Dengue/genetics , Heterozygote , Humans , Immune System , Interferons , Malaria/genetics , Pandemics , Prevalence , Thailand/epidemiology , Thalassemia/epidemiology , Thalassemia/genetics
18.
Orphanet J Rare Dis ; 15(1): 265, 2020 09 25.
Article in English | MEDLINE | ID: covidwho-797723

ABSTRACT

South Asia is the hotspot of beta-thalassemia, with an estimated 200,000 patients whose lives depend on regular blood transfusion. Due to COVID-19 pandemic, many countries have adopted unprecedented lockdown to minimize the spread of transmission. Restriction of nationwide human mobility and fear of COVID-19 infection has put thalassemia patients in a life-threatening situation because of an acute shortage of blood supply. As a public health preparedness strategy during a crisis like COVID-19 pandemic, the plights of thalassemia patients should be considered. Government-sponsored community blood-banks needs to be established or coverage expanded as a safety net for the thalassemia patients in lower- and middle-income countries.


Subject(s)
Coronavirus Infections , Pandemics , Pneumonia, Viral , Thalassemia , Asia , Bangladesh , Betacoronavirus , COVID-19 , Cross-Sectional Studies , Humans , SARS-CoV-2 , Students
19.
Acta Biomed ; 91(3): e2020026, 2020 09 07.
Article in English | MEDLINE | ID: covidwho-761241

ABSTRACT

A review of the literature on COVID-19 pandemic in patients with thalassemias is presented. Globally, the prevalence of COVID-19 among  ß-thalassemia patients seems to be lower than in general population; associated co-morbidities aggravated the severity of  COVID- 19, leading to a poorer prognosis, irrespective of age. A multicenter registry will enhance the understanding of COVID-19 in these patients and will lead to more evidence-based management recommendations.


Subject(s)
Betacoronavirus , Coronavirus Infections/epidemiology , Pandemics , Pneumonia, Viral/epidemiology , Thalassemia/epidemiology , COVID-19 , Comorbidity , Global Health , Humans , Prevalence , SARS-CoV-2
20.
Am J Case Rep ; 21: e925788, 2020 Jul 22.
Article in English | MEDLINE | ID: covidwho-665389

ABSTRACT

BACKGROUND Beta-hemoglobinopathies and glucose-6-phosphate dehydrogenase (G6PD) deficiency are genetic disorders that cause hemolytic anemia when exposed to oxidative stress. Their co-existence is, however, not proven to enhance the severity of anemia. CASE REPORT We report the case of a young man with no known co-morbidities, who came with fever and cough and was diagnosed with COVID-19 pneumonia. He was found to have hemoglobin D thalassemia and G6PD deficiency during further evaluation. Hydroxychloroquine therapy started initially, was discontinued after 3 doses once the G6PD deficiency was diagnosed. His hospital course showed a mild drop in hemoglobin with evidence of hemolysis on peripheral smear. However, the hemoglobin improved without any need for transfusion. CONCLUSIONS Hydroxychloroquine therapy can induce hemolytic crises in patients with underlying G6PD deficiency or hemoglobinopathies and should be avoided or closely monitored. Immediate intervention to stop hydroxychloroquine after 3 doses saved our patient from a major hemolytic crisis. The significance of this case report is that it is the first report that outlines the clinic course of COVID-19 pneumonia in a patient with underlying hemoglobin D disease and G6PD deficiency.


Subject(s)
Betacoronavirus , Coronavirus Infections/complications , Glucosephosphate Dehydrogenase Deficiency/complications , Hemoglobins/metabolism , Pneumonia, Viral/complications , Thalassemia/complications , Asymptomatic Diseases , COVID-19 , Coronavirus Infections/virology , Glucosephosphate Dehydrogenase Deficiency/blood , Humans , Male , Pandemics , Pneumonia, Viral/virology , SARS-CoV-2 , Thalassemia/blood , Young Adult
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