The acute chest syndrome (ACS), a pneumonia-like illness in sickle cell patients, is one of the most frequent causes of their morbidity and hospitalizations. Repeated ACS events may predict the development of chronic lung disease. ACS is reported as a frequent cause of death in these patients. We examine here the incidence and risk factors of ACS in 3,751 patients with sickle cell disease who w...

Background: Recessively inherited genetic disorders such as sickle c thalassemia are commonly encountered in heterozygous and homozygous form in India. These hemolytic disorders cause a high degree of reproductive wastage in vulnerable communities. Inbreeding is usually the mating between two related indivi process of heterosis. Purpose: This study was aimed at finding reproductive outcome in c...

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

S ICKLE CELL-THALASSEMIA DISEASE is known to be a severe or moderately severe type of congenital hemolytic anemia. The clinical and hematologic pictures of this hemoglobinopathy are usually similar to those found in the intermediate type of Cooley’s anemia; however, mild or asymptomatic cases have been reported by various investigators. 6 Singer et al.1 have described four Negro patients with m...

BACKGROUND In sickle cell disease, the quantification of Hb A2 is important for the differential diagnosis between sickle cell anemia (Hb SS) and Hb S/β(0)-thalassemia. OBJECTIVE To determine Hb A2 levels as quantified by high performance liquid chromatography in patients with sickle cell anemia (Hb SS) and with the SC hemoglobinopathy, with or without concomitant alpha thalassemia. METHODS...

Allogeneic cellular gene therapy through hematopoietic stem cell transplantation is the only radical cure for congenital hemoglobinopathies like thalassemia and sickle cell anemia. Persistent mixed hematopoietic chimerism (PMC) has been described in thalassemia and sickle cell anemia. Here, we describe the clinical course of a 6-year-old girl who had received bone marrow transplant for sickle c...

BACKGROUND Sickle cell disease (SCD) is a major health burden in India. The objective of the study was to establish a neonatal screening program and to understand the clinical course of children with SCD in central India. METHODS AND FINDINGS Pregnant mothers were screened for sickle hemoglobin using the solubility test. Babies were screened by high performance liquid chromatography if the mo...

A method for isolating human hemoglobin messenger RNA (mRNA) from bone marrow cells was developed to investigate the molecular basis for the defect in globin synthesis in beta thalassemia. Active mRNA was isolated from the bone marrow cells and peripheral reticulocytes of patients with homozygous beta thalassemia, heterozygous beta thalassemia, sickle cell trait, double heterozygosity for beta ...