OBJECTIVE Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare chan...

Hemoglobinopathies are very common in Greece, the incidence of beta-thalassemia trait being 8% and that of sickle cell trait ranging from 1 to 32% in various districts. In Greek populations, sickle cell disease (SCD) is mainly represented by S-beta thalassemia.

Hematopoietic stem cell transplantation (HSCT) still remains the only definitive cure currently available for patients with thalassemia and sickle cell anemia. Results of transplant in thalassemia and in sickle cell anemia have steadily improved over the last two decades due to improvements in preventive strategies, and effective control of transplant-related complications. From 2004 through 20...

The inherited diseases of hemoglobin have remarkable phenotypic variability because of genetic modifiers necessitating medical intervention at various stages of disease. Genotype–phenotype relationship is crucial in this regard. So three year retrospective study of biochemical pattern of Hemoglobinopathies and Thalassemias and their clinical manifestations was done in a cohort of 689 patients i...

background: until now, trimodal distribution of hbs has been seen by six different studies in the world when associated with alpha-thalassemia with confirmation by corresponding alpha-genotyping studies. the rbc indices reduce as alpha-globin genes reduce in sickle cell trait (sct) patients, which decreases the extent of intra-vascular sickling and thus betters the clinical course of the patien...

Medical advances in the management of patients with sickle cell disease, thalassemia, and other hemolytic anemias have led to significant increases in life expectancy. Improved public health, neonatal screening, parental and patient education, advances in red cell transfusion medicine, iron chelation therapy, penicillin prophylaxis for children, pneumococcal immunization, and hydroxyurea therap...

a Thalassemia modifies the hematologic expression of homozygous sickle cell (55) disease, resulting in increased total hemoglobin and HbA2 and decreased HbF. mean cell volume, reticulocytes. irreversibly sickled cells. and bilirubin levels. The age at which these changes develop in children with 55 disease is unknown. Ascertainment of globin gene status in a large representative sample of child...

Background: The simultaneous existence of two different abnormalities in a gene is referred to double heterozygosity. The most common form of it is the association of thalassemia and sickle cell disease, being termed sickle cell thalassemia. The aim of this research was to study all of the hemoglobinopathies in thalassemia major patients in Lorestan Province. Materials and methods: This st...

Advances in the care of patients with thalassemia, sickle cell disease, and other hemolytic disorders through early detection, education, improvements in red cell transfusion and iron chelation therapy, penicillin prophylaxis, vaccination, and hydroxyurea therapy have led to a significant prolongation of the life expectancy of such patients. As this patient population ages, new chronic complica...

Thalassemia is among the most common genetic disorders worldwide; 4.83 percent of the world’s population carry globin variants, including 1.67 percent of the population who are heterozygous for a -thalassemia and b -thalassemia. In addition, 1.92 percent carry sickle hemoglobin, 0.95 percent carry hemoglobin E, and 0.29 percent carry hemoglobin C. Thus, the worldwide birth rate of people who ar...