Survival and Complications of Beta-Thalassemia in Lebanon

Abstract

β-Thalassemia major is a debilitating disease with a considerable incidence in Lebanon (around 2–3% carriership). The present article describes our experience to this day with 214 patients, emphasizing the survival of β-thalassemia major and development of complications among patients with different parameters. Fifteen deaths were reported. The most common cause of death was heart failure (60%). Patients with a ferritin level of 3,000 ng/ml showed better survival than those with a level >3,000 ng/ml (p < 0.006). In addition, patients with a ferritin level of 1,500 ng/ml showed less complication-free survival than those with a level >1,500 ng/ml (p < 0.024). High level of ferritin (1,500 ng/ml) is associated with increased risk of heart failure. Overall and complication-free survival were statistically different among patients classified according to birth cohort or ferritin level. The Chronic Care Center, a multidisciplinary center located in the suburbs of Beirut, led to an increase in complication-free as well as overall survival. Although patients are being diagnosed earlier and chelation therapy is being initiated at an earlier age, complications due to iron overload still persist. The introduction of new oral iron chelators and better iron overload quantitation methods will most likely modify this picture, and a follow-up study will examine their impact.

Assessment and management of iron overload in β‐thalassaemia major patients during the 21st century: a real‐life experience from the Italian Webthal project

“…Long-term follow-up of b-thalassaemia major (TM) cohorts treated in both developed and developing countries has shown a progressive improvement in life expectancy (Cario et al, 2000;Modell et al, 2000;Borgna-Pignatti et al, 2005;Telfer et al, 2006;Chern et al, 2007;Fung et al, 2007;Charafeddine et al, 2008;Telfer, 2009;Thuret et al, 2010). Part of this transition in the once fatal disease can surely be attributed to the large body of evidence attained by clinical trials and observational studies conducted in the last two decades, which allowed for a remarkable transformation in conventional therapy.…”

Next-generation community genetics for low- and middle-income countries

“…It is estimated that 30% of Muslims and 17% of Christians enter into consanguineous relationships [33]. This leads to a high prevalence and high burden of disease in Lebanon, with carrier rates of 2% to 4% for alpha-thalassemia, 2% to 4% for beta-thalassemia [34], and 0.3% to 30% for sickle cell anemia in Arab countries [32]. It is likely that other autosomal recessive conditions also occur at higher rates than in other countries.…”

Common Complications in Beta-Thalassemia Patients.

“…In the same study, 55 deaths were reported and 60% of them were detected to be caused by heart failure. 23 In the study of Ladis et al, survival at 40 years declined up to 28.9% in the patients whose serum ferritin levels were >4000 µg/L. In the same study, 115 deaths occurred and the leading cause of death was hemosiderosis (76.5%) with 71.3% of cardiac diseases followed by sepsis (7.8%) and AIDS (6.1%).…”