Prevalence of iron overload complications among patients with β-thalassemia major treated at Dubai Thalassemia Centre

Abstract

BACKGROUND AND OBJECTIVESAuthors and team members of the Dubai Thalassemia Centre obtained data on the prevalence of iron overload complications among patients with β-thalassemia major (β-TM) and compared it to international data to improve patient care and evaluate the effectiveness of earlier used treatment modalities. The information obtained is also expected to be useful in genetic counseling.DESIGN AND SETTINGCross-sectional study of all living transfusion-dependent β-TM patients registered at the Thalassemia Centre in Dubai, United Arab Emirates, until the end of 2007 (n=382).PATIENTS AND METHODSDiagnosis of TM was based on clinical history and laboratory confirmation by hemoglobin electrophoresis and DNA testing. All were uniformly treated with desferrioxamine and monitored by serial serum ferritin.RESULTSThe mean (SD) age of patients was 15.4 (7.6) years, with 50.5% males. Mean (SD) serum ferritin was 2597.2 (1976.8) μg/L. The frequency of iron overload complications were as follows: hypogonadism (n=99, 52.7%), hypoparathyroidism (n=40, 10.5%), diabetes mellitus (n=40, 10.5%), hypothyroidism (n=24, 6.5%) and cardiomyopathies (n=7, 1.8%). Hypogonadism was the most common endocrine abnormality in our study and other reported series. However, cardiomyopathies were less prevalent among our patients with higher rates of diabetes and hypoparathyroidism compared to rates reported internationally. Females had statistically significant lower serum ferritin (2530.8 (1931.2), P<.05) with a lower cardiomyopathies rate.CONCLUSIONIron overload related complications among our patients with thalassemia major were different from those reported internationally. Studying the genetic status of patients from our area may uncover the underlying genetic modifiers of iron overload mediated organs injury.

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

“…Among the well-documented complications of β-TM related to iron overload is hypothyroidism, which varies in frequency in different studies worldwide between 4.25% and 10.8%, so our rate of 7.3% is not unexpected. 10 - 14 , 16 , 18 , 28 , 30 , 31 On the other hand, the rate of hypoparathyoidism at 3.3% is less than the rates reported in some other populations of 4.0–10.5%. 10 , 12 , 14 , 18 , 28 , 30 , 31 Diabetes mellitus is another recognized complication and varies in frequency between 2.1% and 26.7%, so our rate of 3.3% is near the lower end of the range.…”

“… 27 The median ferritin in our patients is nearer to figures reported in the UAE and Turkey but is higher than those reported in France and North America and lower than those reported in some Iranian and Egyptian studies. 10 - 14 , 28 To a great extent, this reflects the adequacy of chelation therapy in developed countries compared to some developing countries and is probably also related to the availability and type of chelation administered and literacy of patients or their guardians in different countries. The need to introduce more robust tests for evaluating iron overload, including liver iron content testing and T2* magnetic resonance imaging (MRI) is important.…”

Iron overload in beta thalassemia major patients

“…However lifelong administration of iron chelation regimens has posed challenges to clinicians and patients equally [16]. Poor compliance to chelation therapy and severe iron overload among patients has been reported in both developed and developing countries including United States [17], Pakistan [18], Egypt [19], India [20], Japan [21], Italy [22] and Dubai [23].…”

Hypersensitivity reaction with deferasirox

“…Complications related to iron overload may result in growth retardation, dilated cardiomyopathy, hepatitis, liver cirrhosis and endocrine dysfunction such as diabetes mellitus, hypogonadism, hypothyroidism, hypopitutarism, osteoporosis etc., with cardiac complications being the most common cause of death. [ 1 2 ] Some may also suffer from transfusion-related complications such as HIV and Hepatitis B and C. Survival of individuals with proper transfusion and chelation is beyond 40 years. Iron chelating agents like deferoxamine, deferiprone and deferasirox have dramatically improved the outlook in beta thalassemia.…”