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Thalassemias are inherited blood disorders. Thalassemias cause the body to make fewer healthy red blood cells and less hemoglobin than normal. Hemoglobin is an iron-rich protein in red blood cells.

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Thalassaemia is a group of genetic blood disorders. Blood transfusion is needed to treat severe forms of the condition, but this results in a high level of iron in the body. This excess iron is usually removed by drugs through 'chelation therapy'. Other less usual treatments are bone marrow transplantation, which is restricted by age, and hydroxyurea, a drug designed to raise the level of foetal haemoglobin and reduce anaemia. Psychological support would seem suitable to manage thalassaemia which is a chronic disease with a considerable role for self‐management. We were not able to find any trials to include in this review. There are some reports that teaching people about their condition improves adherence to chelation therapy. However, from the information currently available, we can not recommend the use of specific psychological therapies in thalassaemia. We believe there is a need for large trials to assess the value of psychological therapies for thalassaemia.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: March 6, 2014

We looked for evidence to show whether identifying people who are carriers for thalassaemia, sickle cell disease, cystic fibrosis, or Tay‐Sachs disease, before pregnancy leads to improving reproductive choice and pregnancy outcomes.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: August 12, 2015

We reviewed the evidence about the effect and safety of different types of allogeneic stem cell transplantation in people with severe transfusion‐dependant ß‐thalassaemia major or ß0/+‐thalassaemia variants requiring chronic blood transfusion.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: November 30, 2016

We reviewed the evidence comparing the removal of the spleen (splenectomy) to other treatments (such as blood transfusion and chelation (removal of excess iron) in people with thalassaemia major and intermedia.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: June 14, 2016

Zinc is an essential micronutrient, which is needed so that the immune system works at its best and helps the body fight off infection. People may not get enough zinc from food alone. Researchers have therefore looked at zinc supplements as a way of reducing anaemia and preventing infections and complications. The review authors searched the medical literature for randomised controlled studies in which people with sickle cell disease or thalassaemia received either zinc supplements or no supplements. We included nine trials in the review (459 participants). In people with thalassaemia, there is no evidence to indicate any benefit of zinc supplements on serum zinc level. However, there was an improvement in height in those who received the supplements. There is mixed evidence on the benefit of using zinc supplements in people with sickle cell disease. For instance, there is evidence that when supplements are given for one year the serum zinc levels increased; however, haemoglobin levels and body mass index did not differ significantly between groups. We also found that people with sickle cell disease who received zinc supplements (at both three months and at one year) had fewer sickle cell crises and infections. However, given that the total number of trials is small, these results should be treated with caution.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: June 28, 2013

In thalassaemia sometimes the body cannot produce enough haemoglobin. Regular blood transfusions can manage this, but may lead to excess iron in the body, which, if not removed, may damage major organs. Iron chelation therapy removes excess iron; one common chelator is deferiprone. Questions exist about whether deferiprone is as good and safe as the most widely‐used iron chelator, desferrioxamine. Desferrioxamine is administered by a needle under the skin or into a vein, and was the standard monotherapy for 20 years.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: August 21, 2013

Haemoglobin carries oxygen in the blood. In thalassaemia, a genetic disease, sometimes the body cannot produce enough haemoglobin. This can be managed by receiving regular blood transfusions, but may lead to excess iron in the body which must be removed to prevent organ damage.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: August 21, 2013

We reviewed the evidence on the effects and safety of different treatments of osteoporosis in people with beta‐thalassaemia.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: March 10, 2016

Thalassaemia is a hereditary anaemia due to a defect in the production of haemoglobin. Regular red blood cell transfusions are needed, particularly for the severe form of the disease, thalassaemia major. This results in iron overload. Since the human body has no means of actively getting rid of excessive iron, drug treatment (iron‐chelating drugs) is needed. Several years ago, a newer oral iron chelator, deferasirox, was introduced.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: August 15, 2017

We reviewed the evidence about the effect of treating people with thalassaemia with growth hormones.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: 2017

Bibliographic details: Xu Y, Xia SJ, Zhang SH, Jiang H.  Iron chelation therapy in beta-thalassemia major: a systematic review with meta-analysis. Chinese Pharmaceutical Journal 2013; 48(21): 1875-1880 Available from: http://caod.oriprobe.com/articles/40548660/Iron_Chelation_Therapy_in_Beta_Thalassemia_Major_A_Systematic_Review_w.htm

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2013

We wanted to find out if giving hydroxyurea to people with non‐transfusion dependent beta thalassaemia would reduce the need for blood transfusion.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: October 18, 2016

We reviewed the evidence about the effects of prophylactic antibiotic regimens for preventing pneumococcal infection in children with sickle cell disease (SCD). This is an updated version of a previously published Cochrane Review.

Cochrane Database of Systematic Reviews: Plain Language Summaries [Internet] - John Wiley & Sons, Ltd.

Version: October 10, 2017

The effectiveness of deferoxamine (DFO), deferiprone (DFP), or deferasirox (DFX) in thalassemia major was assessed. Outcomes were reported as means±SD, mean differences with 95% CI, or standardized mean differences. Statistical heterogeneity was tested using χ2 (Q) and I2. Sources of bias and Grading of Recommendations Assessment, Development and Evaluation system (GRADE) were considered. Overall, 1520 patients were included. Only 7.4% of trials were free of bias. Overall measurements suggest low trial quality (GRADE). The meta-analysis suggests lower final liver iron concentrations during associated versus monotherapy treatment (p<0.0001), increases in serum ferritin levels during DFX 5, 10, and 20 mg/kg versus DFO-treated groups (p<0.00001, p<0.00001, and p=0.002, respectively), but no statistically significant difference during DFX 30 mg/kg versus DFO (p=0.70), no statistically significant variations in heart T2* signal during associated or sequential versus mono-therapy treatment (p=0.46 and p=0.14, respectively), increases in urinary iron excretion during associated or sequential versus monotherapy treatment (p=0.008 and p=0.02, respectively), and improved ejection fraction during associated or sequential versus monotherapy treatment (p=0.01 and p<0.00001, respectively). These findings do not support any specific chelation treatment. The literature shows risks of bias, and additional larger and longer trials are needed.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2011

OBJECTIVE: A meta-analysis was conducted to investigate the efficacy and safety of three main iron chelators, namely, deferoxamine (DFO), deferiprone (DFP) and deferasirox (DFX) for thalassemia major (TM) patients.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2013

β-Thalassemia major (β-TM) patients require life-long blood transfusions, resulting in iron overload with multi-organ morbidity and mortality. Evidence from small randomized controlled trials (RCTs) published to date for deferiprone (DFP) monotherapy or in combination with deferoxamine (DFO) is unclear. We summarized evidence on the efficacy of DFP monotherapy compared to DFO, and DFP-DFO combination therapy compared to DFP or DFO monotherapy in chronically transfused β-TM. We searched four electronic databases and examined the grey literature. Two authors independently assessed trial quality and extracted data. We calculated the relative risk for dichotomous outcomes and mean difference (MD) for continuous outcomes. We identified 15 RCTs (1003 participants) that met the inclusion criteria. Deferiprone was more efficacious than DFO in improving cardiac ejection fraction [MD 2.88, 95% CI (95% confidence interval) 1.12 to 4.64, p = 0.001) and endocrine dysfunction (MD 0.09, 95% CI 0.08 to 0.10, p < 0.00001). The DFP-DFO combination therapy was more efficacious than DFP or DFO monotherapy in improving cardiac ejection fraction (MD 5.67, 95% CI 1.32 to 10.02, p = 0.008). There was no significant difference in all other outcomes examined. Meta-analysis on changes in myocardial iron content was not possible due to differences in data presentation. The quality of evidence for all outcomes was low. There is currently insufficient evidence to show that DFP is superior to DFO in the treatment of iron overload. The use of DFP must be weighed against the potential side-effects, patient compliance and preference. Large RCTs with clinically relevant outcomes are required.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2014

Hydroxyurea (HU) is being used for patients with transfusion-dependent β-thalassemia major (β-TM) as well as non transfusion-dependent β-TM. As controversy exists regarding efficacy and safety of HU, we searched the published literature on efficacy, effectiveness and toxicity of HU in patients with β-TM. The research sources we used were: Medline, SID, PubMed, Scopus, Request, Web of Knowledge, Springer, Ovid, Cochrane searched up to October 2012. Using search terms sensitive to studies of clinical trials combined with searches on terms related to thalassemia and HU. We selected studies on randomized trials, quasi experimental trials (before and after design), case reports (with 1-5 cases), side effect studies in patients with β-TM, studies related to the mechanism of action and toxicity when used in patients with other hemoglobinopathies. We researched studies in English and Persian. Eligible articles were reviewed by two independent reviewers. Patient's characteristics, duration of trial, outcome and side effects were extracted. The main outcomes were synthesized under a random-effects model. Heterogeneity was assessed using the Q statistic, Tau(2) and I(2). Subgroup analyses were performed and the statistics data (STATA) software used. More than 500 articles were reviewed. No randomized clinical trial was found. Seventeen trials with before and after designs were found, 16 case reports (1-5 cases), 19 articles for mechanism of action and 16 studies for side effects were published from 1969 to October 2012. Hemoglobin levels after treatment showed modest but significant increase in non transfusion-dependent β-TM (p < 0.0001) and in transfusion-dependent β-TM (p < 0.0001).

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2014

The authors concluded that zoledronate was a promising bisphosphonate that improved bone mineral density in thalassaemia-induced osteoporosis, but that more research is required to understand its potential adverse effects. The review suffered from poor reporting and had potential methodological weaknesses, including the uncertain quality of included trials, which limits the reliability of the authors’ conclusion.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2010

This review assessed the effect of current anti-hepatitis C virus (HCV) treatment in polytransfused thalassaemic patients and concluded that patients infected with Genotype 1 HCV benefited from addition of ribavirin to their therapeutic regimen; although it may increase transfusion need, it did not increase adverse event rates. For methodological reasons the validity of this conclusion is unclear.

Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet] - Centre for Reviews and Dissemination (UK).

Version: 2010

To assist the United States Preventive Services Task Force (USPSTF) in updating its 1996 recommendation on screening for hemoglobinopathies in newborns, staff at the Agency for Healthcare Research and Quality (AHRQ) performed a literature search and consulted with subject area experts. The goal of this targeted review was to find new, high-quality evidence regarding the benefits and potential harms of screening for hemoglobinopathies in newborns. Sixty-nine studies were initially identified. The literature search methodology is described in Appendix I. One systematic review of benefits of screening, one systematic review of benefits of penicillin prophylaxis, and three articles about potential harms met inclusion criteria and are discussed in the following review.

Evidence Syntheses - Agency for Healthcare Research and Quality (US).

Version: September 2007

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