Evaluating Health-Related Quality of Life (HRQoL) in Iraqi Adult and Pediatric Patients with Beta-Thalassemia Major Using Two Different Iron Chelation Therapies

Shawkat, Ali Jalal; Jwaid, Ahmed Hamed; Awad, Ghada Marzouq

doi:10.31351/vol28iss1pp44-52

Cited by 7 publications

(6 citation statements)

References 28 publications

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“…Splenomegaly increases the need for blood transfusions. Splenectomy is indicated when hypersplenism leads to an increased requirement for blood transfusions and interferes with effective iron control through chelation therapy [18,19] .…”

Section: Discussionmentioning

confidence: 99%

Investigation of JAK2 V617F mutation prevalence in a sample of Iraqi patients with Beta thalassemia major

Ameer¹,

Ahmed²

2023

Int. J. Clin. Diagn. Pathol.

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Introduction: β-thalassemia major is a common inherited hemoglobin disorder worldwide, characterized by shortened red cell survival, defective hemoglobin synthesis, resulting in hemolytic anemia, ineffective erythropoiesis, severe anaemia, hypoxia, and hepatosplenomegaly. This study aimed to evaluate the occurrence of the JAK2 V617F mutation and JAK2 polymorphism in βthalassemia patients compared to non-β-thalassemia controls. Additionally, the relationship between JAK2 mutations/polymorphisms and specific clinical and hematological parameters was investigated. Methods: This was a case-control study involving 71 participants, comprising 51 individuals with βthalassemia major (patient group) and 20 healthy individuals matched for age and gender (control group). Hematological parameters were measured using an automated analyzer. Venous blood samples were collected with EDTA anticoagulant and stored at 4 °C for subsequent detection of JAK2 V617F mutation using DNA sequencing methods. Results: Analysis of β-thalassemia patients receiving regular blood transfusions revealed significantly lower levels of red blood cells (RBCs), packed cell volume (PCV), hemoglobin (Hb), mean corpuscular volume (MCV), mean corpuscular hemoglobin (MCH), and higher levels of white blood cells (WBC) and ferritin compared to the control group. However, the JAK2 V617F mutation was not detected in any patients or controls. The most common genotype observed in β-thalassemia patients was TC (52.94%), followed by the TT genotype, but this did not show significant associations with clinical and hematological parameters. However, there were significant differences in the frequencies of genotypes of the rs12343867 polymorphism between patients and controls. Conclusion:The study did not find evidence supporting the role of JAK2 V617F mutation in the development or clinical course of β-thalassemia. Furthermore, the mutation was not detected in the normal subjects either. The frequency of JAK2 gene polymorphism did not significantly differ between thalassemia patients and normal subjects, and different genotypes of the gene polymorphism did not show associations with demographic, clinical, or hematological parameters.

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Section: Discussionmentioning

confidence: 99%

Investigation of JAK2 V617F mutation prevalence in a sample of Iraqi patients with Beta thalassemia major

Ameer¹,

Ahmed²

2023

Int. J. Clin. Diagn. Pathol.

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“…Studies indicated that ICT did not provide substantial improvements in quality of life, but that the economic burden of long-term treatment with ICT was high. Patients also continued to experience endocrine dysfunction despite lifelong treatment with ICT [ 17 , 19 , 20 , 25 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 ].…”

Section: Discussionmentioning

confidence: 99%

Relationship between Serum Ferritin and Outcomes in β-Thalassemia: A Systematic Literature Review

Shah

Huey

Deshpande

et al. 2022

JCM

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Among the difficulties of living with β-thalassemia, patients frequently require blood transfusions and experience iron overload. As serum ferritin (SF) provides an indication of potential iron overload, we conducted a systematic literature review (SLR) to assess whether SF levels are associated with clinical and economic burden and patient-reported outcomes (PROs). The SLR was conducted on 23 April 2020 and followed by analysis of the literature. Dual-screening was performed at the title, abstract, and full-text levels using predefined inclusion and exclusion criteria. Ten studies identified by the SLR were eligible for inclusion in the analysis. Seven studies were conducted in Europe, and most were prospective or retrospective in design. The patient populations had a median age of 20.7–42.6 years, with a percentage of men of 38–80%. Sparse data were found on the correlation between SF levels and mortality, and hepatic, skeletal, and cardiac complications; however, in general, higher SF levels were associated with worsened outcomes. The bulk of the evidence reported on the significant association between higher SF levels and endocrine dysfunction in its many presentations, including a 14-fold increase in the risk of diabetes for patients with persistently elevated SF levels. No studies reporting data on PROs or economic burden were identified by the SLR. SF levels provide another option for prognostic assessment to predict a range of clinical outcomes in patients with β-thalassemia.

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“…Numerous transfusion treatments have been projected over the years; however, the utmost extensively conventional goal at pre-transfusion hemoglobin near to 9 to 10 g/dl as well as a post-transfusion level of hemoglobin should be 13 to 14 g/dl. This inhibits impairment of organs, retardation of growth as well as malformations of bones which might lead to normal quality and activity of life (Shawkat et al, 2019). The frequency of blood transfusion depends on numerous causes such as the level of hematocrit and Hb as well as the weight of the patient (Taher & Cappellini, 2018).…”

Section: Blood Transfusionmentioning

confidence: 99%

Current status of beta‐thalassemia and its treatment strategies

Ali

Shakir

Khan

et al. 2021

Molec Gen & Gen Med

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Evaluating Health-Related Quality of Life (HRQoL) in Iraqi Adult and Pediatric Patients with Beta-Thalassemia Major Using Two Different Iron Chelation Therapies

Abstract:

Cited by 7 publications

References 28 publications

Investigation of JAK2 V617F mutation prevalence in a sample of Iraqi patients with Beta thalassemia major

Investigation of JAK2 V617F mutation prevalence in a sample of Iraqi patients with Beta thalassemia major

Relationship between Serum Ferritin and Outcomes in β-Thalassemia: A Systematic Literature Review

Current status of beta‐thalassemia and its treatment strategies

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