Sickle cell anemia was first described by Herrick in 1910. Pioneering studies by Pauling et al. established that Sickle Cell Disease (SCD) results from a defect in the hemoglobin molecule. 1 The sickle mutation was characterized several years later by Ingram et al. as a glutamine-to valine substitution at the sixth residue of the beta globin polypeptide. Homozygosity for the sickle mutation (i....

The only curative treatment approach for patients with sickle cell anemia is allogeneic stem cell transplantation. In this article we will review the published data about stem cell transplantation in patients with sickle cell disease and the small Brazilian experience in this field. The possible indications for stem cell patients will be discussed. Rev. bras. hematol. hemoter. 2007;29(3):327-330.

OBJECTIVE To investigate orbital vascular resistance by Doppler velocimetry in patients with steady-state sickle cell anemia, as well as to characterize its relationship with biomarkers of hemolysis. MATERIALS AND METHODS This was a cross-sectional study of two groups: 71 outpatients with sickle cell anemia; and 32 age- and gendermatched, healthy subjects (control group). All participants und...

OBJECTIVE The purpose of this study was to determine the impact of mean platelet volume (MPV) on the frequency and severity of vaso-occlusive and cerebrovascular events in patients with sickle cell anemia (SCA). METHODS The 238 cases diagnosed with SCA were evaluated retrospectively with respect to the occurrence of painful crisis for the previous year. The incidence, severity and type of the...

BACKGROUND The etiology of stroke, a severe complication of sickle cell anemia, involves inflammatory processes. However, the pathogenetic mechanisms are unknown. The aim of this study was to evaluate the influence of interleukin-10 polymorphisms and haplotypes on the risk of acute cerebral ischemia and high-risk transcranial Doppler in 395 children with sickle cell anemia from the state of Min...

the restriction enzyme ava ii detects the base change of the intervening sequence ii (ivs ii) which is used as one of the markers of β-globin gene polymorphism. this study was conducted to determine the frequency of the ava ii site on the β-globin gene among normal people and patients with sickle cell syndrome (scs) in iran. dna fragments containing the ivs ii region of the β-globin gene from 3...

INTRODUCTION Sickle cell anemia and thalassemia result in impaired bone health in both adults and youths. Children with other types of chronic hemolytic anemia may also display impaired bone health. STUDY DESIGN To assess bone health in pediatric patients with chronic hemolytic anemia, a cross-sectional study was conducted involving 45 patients with different forms of hemolytic anemia (i.e., ...

This study examined the prevalence of high blood pressure, heart disease, and medical diagnoses in relation to blood disorders, among 6,329 adolescent students (age 15 to 18 years) who reside in the United Arab Emirates (UAE). Findings indicated that the overall prevalence of high blood pressure and heart disease was 1.8% and 1.3%, respectively. Overall, the prevalence for thalassemia, sickle-c...

This study aimed to to determine the prevalence of Sickle cell diseases and trait and high hemoglobin (Hb) A2 βthalassemia in the province of Makkah among patients of Al-Noor Specialist hospital, Makkah. Retrospective studying the results of 620 requested saudi patients (256 male and 364 female) suffer from Anemia for gel electrophoresis. Covering the period between 1 January 2012 to 30 Decembe...

Experiments were carried out to test the hypothesis that the differences between the surfaces of erythrocytes from normal and sickle cell patients are reflected in the degree of attachment to the capillary lining. An assay was used that measured the number of 51Cr-labeled erythrocytes (normal or sickle) attaching to a monolayer of endothelium cultured from calf aortas. Under these conditions, e...