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      Complications and Challenges in the Management of Iraqi Patients with β-Thalassemia Major: A Single-center Experience

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          Abstract

          Objectives

          We sought to assess the complications and challenges facing the management of β-thalassemia major (β-TM) in Iraq.

          Methods

          A total of 150 consecutive patients with β-TM who were registered at a main thalassemia center in Northern Iraq were enrolled in the study. The patients had their records reviewed, were clinically evaluated, and investigated for various complications.

          Results

          Our patient cohort had a median age of 13 years (range: 1–35 years) and a male to female ratio of 1:1.2. Their median serum ferritin was 2762 µg/L, all were on regular transfusions, 94.7% were on chelation therapy, and 38.0% were splenectomized. Pre-transfusion hemoglobin levels were 3 9.0 g/dL in 38.7% of the patients. Short stature was encountered in 33.9% of those aged ≤ 20 years, and skeletal changes were noted in 50.7%. Iron overload associated complications, including hypogonadism, hypothyroidism, hypoparathyroidism, diabetes mellitus, and heart failure, were encountered in 52.8%, 7.3%, 3.3%, 3.3%, and 2.7%, respectively. Hepatitis C virus (HCV) antibodies were detectable in 35.3%, while HIV antibodies and hepatitis B surface antigen were not detectable in any. Patients with diabetes mellitus, heart failure, HCV antibodies, and hypoparathyroidism were significantly older than those without these complications. Hypogonadism was the only complication associated with significantly higher serum ferritin levels. Hypogonadism, heart failure, HCV antibodies, and diabetes were significantly more frequent among the splenectomized patients.

          Conclusions

          The management of β-TM in this cohort of Iraqi patients is still suboptimal, and the need to ensure timely transfusions and optimize chelation, as well as a more robust iron overload assessment, should be underscored.

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          Most cited references31

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          The epidemiology of heart failure: The Framingham Study

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            Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine.

            Seven Italian centers reported data on survival, causes of death and appearance of complications in patients with thalassemia major. The interactions between gender, birth cohort, complications, and ferritin on survival and complications were analyzed. Survival after the first decade was studied for 977 patients born since 1960 whereas survival since birth and complication appearance was studied for the 720 patients born after 1970. Better survival was demonstrated for patients born in more recent years (p<0.00005) and for females (p=0.0003); 68% of the patients are alive at the age of 35 years. In the entire population 67% of the deaths were due to heart disease. There was a significant association between birth cohort and complication-free survival (p<0.0005). The prevalence of complications was: heart failure 6.8%, arrhythmia 5.7%, hypogonadism 54.7%, hypothyroidism 10.8%, diabetes 6.4%, HIV infection 1.7%, and thrombosis 1.1%. Lower ferritin levels were associated with a lower probability of heart failure (hazard ratio =3.35, p<0.005) and with prolonged survival (hazard ratio = 2.45, p<0.005), using a cut-off as low as 1,000 ng/mL. Survival and complication-free survival of patients with thalassemia major continue to improve, especially for female patients born shortly before or after the availability of iron chelation.
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              Complications of beta-thalassemia major in North America.

              Treatment of patients with beta-thalassemia major has improved dramatically during the past 40 years; however, the current clinical status of these patients remains poorly characterized. We performed a cross-sectional study of 342 patients in the Registry of the National Institutes of Health-sponsored Thalassemia Clinical Research Network. Evidence of hepatitis C exposure was present in 35% of tested patients, was associated with age, and had a rate of spontaneous viral clearance of 33%. Ferritin levels ranged from 147 to 11 010 ng/mL (median, 1696 ng/mL). Median hepatic iron content was 7.8 mg/g dry weight and 23% of patients had values of 15 mg/g dry weight or higher. No patients 15 years or younger and 5% of patients aged 16 to 24 years had heart disease requiring medication. Ten percent had cirrhosis on biopsy. Endocrinologic complications were common among adults. Seventy-four (22%) patients had recent implantable central venous access devices (CVADs) placed. Among 80 episodes of bacteremia in 38 patients, 90% were attributable to the CVAD. Among 330 patients who had received deferoxamine chelation therapy, 224 (68%) reported no complications. We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with beta-thalassemia.
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                Author and article information

                Journal
                Oman Med J
                Oman Med J
                OMJ
                Oman Medical Journal
                OMJ
                1999-768X
                2070-5204
                27 July 2020
                July 2020
                : 35
                : 4
                : e152
                Affiliations
                [1 ]Department of Hematology, Azadi Teaching Hospital, Duhok, Iraq
                [2 ]Department of Pathology, College of Medicine, University of Duhok, Duhok, Iraq
                Author notes
                [* ]Corresponding author: nallawi@ 123456yahoo.com
                Article
                OMJ-35-04-1900145
                10.5001/omj.2020.72
                7383834
                a5a69080-9355-4f0c-8749-65b61a989cfb
                The OMJ is Published Bimonthly and Copyrighted 2020 by the OMSB.

                This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial (CC BY-NC) 4.0 License. http://creativecommons.org/licenses/by-nc/4.0/

                History
                : 07 September 2019
                : 09 February 2020
                Categories
                Original Article
                Original Article

                beta-thalassemia,thalassemia,hepatitis c antibodies,iraq

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