Sickle cell disease: the Lebanese experience

Abstract

Summary Sickle cell disease (SCD), the commonest single gene disorder worldwide, is an inherited disease that has different clinical and hematological manifestations in different populations. The objective of this study is to describe the characteristics of the Lebanese SCD population. This was a retrospective study that included information on 387 patients with either sickle cell anemia (SS) or sickle beta‐thalassemia (ST). The mean (±SD) age was 17.9 years (±12.5), and the mean (±SD) follow‐up was 9.3 ± 6.9 years. Fifty percent of the patients were males and SS/ST distribution was 3 : 1. The disease was clustered in two geographic areas in North and South Lebanon. Nearly, all patients were Muslims and 56% were the offspring of consanguineous parents. The prevalence of splenomegaly beyond 6 years of age among SS patients was 28.9%. The prevalence rates of stroke, leg ulcers and priapism were 4.1%, 1.4%, and 0.8%, respectively. Comparing the SS and the ST patients, there were no statistically significant differences in the prevalence of all clinical manifestations except for splenomegaly (SS: 28.9%, ST: 54.9%, P‐value < 0.001) and splenectomy (SS: 16.1%, ST: 35.7%, P‐value < 0.001). In contrast to Northern American populations and similar to some Mediterranean populations, Lebanese SCD patients have a higher prevalence of persistent splenomegaly. The relatively low incidence of thrombotic complications deserves further investigation. The study’s limitations include those of any other retrospective study and the fact that not all Lebanese centers caring for inherited hemoglobin disorders were included. However, the results of this first large scale national survey indicate that preventive efforts should target the Northern and Southern regions of Lebanon to decrease the number of new off springs afflicted with this disease similar to what has been successfully achieved with Thalassemia, another hemoglobinopathy that is highly prevalent in the country.

The Daily Experiences of Adolescents in Lebanon With Sickle Cell Disease

“…These estimates may be lower than the actual incidence because of the early discharge of newborns and lack of newborn screening at many institutions. Compared with international data, the carrier rate of sickle cell and other hemoglobin variants in Lebanon is similar to Brazil (approximately 2%) but almost twice that of Italy (1.2%) and lower than Saudi Arabia (1.3% to 21.3%) and Bahrain (11.2% to 16.4%; Haber et al, 2010;Inati et al, 2007;Jastaniah, 2011;Makhoul et al, 2005).…”

“…CCCL is affiliated with St. Jude's Children's Research Hospital in Memphis, Tennessee, and works in coordination with the AUBMC. The exact number of children affected with SCD in Lebanon is unknown but is estimated to be around 1,000, with the majority being Muslim and residing in the northern and southern regions of the country (Inati et al, 2007). The comprehensive sickle cell program at AUBMC provides state-of-the-art care to patients with this genetic disorder.…”

“…The term refers to a collection of autosomal-recessive genetic disorders characterized by the hemoglobin S (HbS) variant of the b-globin gene that is resistant to malaria development and in which more than 50% the hemoglobin-b gene is replaced with HbS (El-Hazmi, Al-Hamzi, & Warsy, 2011;Saraf et al, 2014). SCD affects 1 in 500 African Americans and more than 30 million people worldwide, with predominance in populations of the sub-Sahara, India, and the Middle East (Inati et al, 2007;Khoriaty et al, 2014;McGann, 2014). There are different variants of the b-globin gene in SCD, including but not limited to sickle cell anemia, sickle-hemoglobin C disease, sickle beta-plus thalassemia, and sickle beta-zero thalassemia, with prevalence of certain variants types in particular ethnic groups (El-Hazmi et al, 2011;Saraf et al, 2014).…”

Sickle cell disease in Middle East Arab countries

“…The disease was shown to be clustered in two geographic areas in North and South Lebanon and nearly all patients were Muslims74. The disease was severe and the major haplotype was the Benin haplotype.…”

Prenatal Molecular Diagnosis of β-Thalassemia and Sickle Cell Anemia in the Syrian Population

“…Hb S was present in 20.0% of the 55 couples included in this study, and it was the only abnormal Hb found. This high frequency is often reported in many parts of Africa, India, the Middle East, the Mediterranean area, and parts of Turkey (3,(34)(35)(36). In this study, all couples who were diagnosed as carriers of the Hb S mutation originated from the Syrian coast or the southern regions of Syria.…”