Morbidities and Mortality in Transfusion-Dependent Beta-Thalassemia Patients (Single-Center Experience)

Mokhtar, Galila M.; Gadallah, Mohsen; Sherif, Nayera H.K. El; Ali, Heba

doi:10.3109/08880018.2012.752054

Cited by 42 publications

(42 citation statements)

References 35 publications

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“…Mokhtar and associates [53] reported the presence of cardiovascular morbidities in 41.3% of β-thalassemia patients with cardiomyopathy being the most frequent (6.9%), followed by arrhythmia (1.6%) and PH (0.4%). Another study done by Bejaoui and Guirat [54] showed that cardiac disorders occurred in 19.8% of the studied β-TM patients with higher incidence of PH (12.5%) and heart failure (39%) being the major leading cause of death.…”

Section: Discussionmentioning

confidence: 99%

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

et al. 2017

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Background: Ischemia-modified albumin (IMA) is an altered type of serum albumin that forms under conditions of oxidative stress and an independent predictor of major adverse cardiovascular events. Objectives: To measure the levels of IMA in 45 children and adolescents with β-thalassemia major (β-TM) compared with 30 healthy controls and assess its relation to lipid peroxidation, vascular complications and subclinical atherosclerosis. Methods: β-TM patients without symptoms of heart disease were studied focusing on transfusion history, chelation therapy, serum ferritin, malondialdehyde (MDA) and IMA levels. Echocardiography was performed and carotid intima media thickness (CIMT) was assessed. Results: IMA and MDA levels were significantly higher in β-TM patients compared with controls (p < 0.001). IMA was higher among patients with heart disease, pulmonary hypertension risk and serum ferritin ≥2500 µg/l than those without. TM patients compliant to chelation had significantly lower IMA levels. IMA levels were positively correlated to MDA and CIMT while negatively correlated to ejection fraction and fractional shortening. Conclusion: Our results highlight the role of oxidative stress in the pathophysiology of vascular complications in thalassemia. IMA could be useful for screening of β-TM patients at risk of cardiopulmonary complications and atherosclerosis because its alteration occurs in early subclinical disease.

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Section: Discussionmentioning

confidence: 99%

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

et al. 2017

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“…In a retrospective study of patients with transfusion‐dependent β‐thalassemia, mortality was significantly higher among patients who were less than 20% compliant than those with better compliance ( P < .05) . Noncompliance was most common among those taking deferoxamine compared with deferiprone and deferasirox.…”

Section: Resultsmentioning

confidence: 71%

“…A total of 18 individual papers describing 15 different studies were published between 2000 and 2017, and are summarized in Table . These studies included 7189 patients; however, some patients likely were counted more than once since the multicenter studies from Borgna‐Pignatti et al, although distinct, drew on the same patient populations in Italy .…”

Section: Resultsmentioning

confidence: 99%

“…Only 1 of the retrospective/longitudinal studies compared survival outcomes in β‐thalassemia patients according to the ICT received; a second study compared monotherapy to combination therapy . The remaining studies investigated survival outcomes according to various baseline and treatment characteristics: 1 investigated factors associated with death in a case‐control study; 4 studied outcomes according to compliance with or duration of ICT; 4 compared survival outcomes according to period of treatment (i.e., before or after availability of different iron chelation treatments); 1 measured survival in patients with thalassemia major and thalassemia intermedia before and after introduction of deferoxamine; and 1 measured rates of death in hepatitis C virus (HCV)‐infected and noninfected patients receiving ICT with deferoxamine …”

Section: Resultsmentioning

confidence: 99%

“…These studies have demonstrated that treatment outcomes have improved significantly over time with the progressive introduction of new and improved ICTs, including oral treatments and combination therapy . The effectiveness of iron chelation has also been demonstrated in studies investigating treatment compliance where survival is much better in patients with good compliance with ICT . Importantly, compliance has been much improved by the introduction of oral agents such as deferiprone and deferasirox .…”

Section: Discussionmentioning

confidence: 99%

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The effect of iron chelation therapy on overall survival in sickle cell disease and β‐thalassemia: A systematic review

et al. 2018

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Red blood cell transfusions have become standard of care for the prevention of life-threatening anemia in patients with β-thalassemia and sickle cell disease (SCD). However, frequent transfusions can lead to accumulation of iron that can result in liver cirrhosis, diabetes mellitus, arthritis, arrhythmias, cardiomyopathy, heart failure, and hypogonadotropic hypogonadism. Iron chelation therapy has been shown to reduce serum ferritin levels and liver iron content, but limitations of trial design have prevented any demonstration of improved survival. The objective of this systematic review was to investigate the impact of iron chelation therapy on overall and event-free survival in patients with β-thalassemia and SCD. Eighteen articles discussing survival in β-thalassemia and 3 in SCD were identified. Overall iron chelation therapy resulted in better overall survival, especially if it is instituted early and compliance is maintained. Comparative studies did not show any significant differences between available iron chelation agents, although there is evidence that deferiprone is better tolerated than deferoxamine and that compliance is more readily maintained with the newer oral drugs, deferiprone and deferasirox. Iron chelation therapy, particularly the second-generation oral agents, appears to be associated with improved overall and event-free survival in transfusion-dependent patients with β-thalassemia and patients with SCD.

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The burden of psychiatric illnesses in adult patients with beta-thalassemia: a 5-year nationwide inpatient evaluation in the United States

et al. 2018

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Morbidities and Mortality in Transfusion-Dependent Beta-Thalassemia Patients (Single-Center Experience)

Abstract: In the past 10 years, the survival and morbidity rates in our center have markedly improved as a result of regular blood transfusion, new iron chelators, and better compliance of the patients.

Cited by 42 publications

References 35 publications

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

Ischemia-modified albumin as a marker of vascular dysfunction and subclinical atherosclerosis in β-thalassemia major

The effect of iron chelation therapy on overall survival in sickle cell disease and β‐thalassemia: A systematic review

The burden of psychiatric illnesses in adult patients with beta-thalassemia: a 5-year nationwide inpatient evaluation in the United States

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