Next-generation ektacytometry provided by the osmoscan module of Laser Optical Rotational Red Cell Analyser (LoRRca) MaxSis is, so far, one best complementary diagnostic tools for congenital rare anaemias due to red blood cell defects. Osmotic gradient (OGE) is currently considered gold standard diagnosis membrane disorders, especially hereditary spherocytosis (HS). Impairment deformability, le...

CONTEXT AND OBJECTIVE Transcranial Doppler (TCD) detects stroke risk among children with sickle cell anemia (SCA). Our aim was to evaluate TCD findings in patients with different sickle cell disease (SCD) genotypes and correlate the time-averaged maximum mean (TAMM) velocity with hematological characteristics. DESIGN AND SETTING Cross-sectional analytical study in the Pediatric Hematology sec...

We have determined the frequency of deletional a-thalassemia in black populations in the USA and Africa that harbor sickle cell anemia. In normals. the frequency of the chromosome bearing a deletion of one of the two normal a gene loci. designated ( -a). ranged from 0.12 to 0.16, and in sickle trait subjects. the frequency ranged from 0.1 8 to 0.20. By contrast. in sickle cell anemia subjects. ...

Approximately 100,000 Americans have sickle cell disease (SCD), a group of recessively inherited red blood cell disorders characterized by abnormal hemoglobin, called hemoglobin S or sickle hemoglobin, in the red blood cells. Persons with hemoglobin SS or hemoglobin Sß0 thalassemia, also known as sickle cell anemia (SCA), have the most severe form of SCD. Hemoglobin SC disease and hemoglobin Sß...

Background Sickle cell patients suffer from many physical, psychological, and social problems that can affect their quality of life. To deal with this chronic condition and manage their disease and prevent complications associated with the disease, they must learn skills and behaviours. The aim of this study was to determine the effectiveness of self-management programs on quality of life in t...

Sickle cell disease (SCD) is a genetic disorder belonging to a group characterized by the predominance of abnormal hemoglobin S (Hb S). Under conditions of deoxygenation, Hb S polymerizes causing vaso-occlusion and tissue injury. Clinical manifestations occur in the course of the disease and infections can also affect patients throughout their lives (1,2). Even though the incidence varies, sick...

The globally widespread single-gene disorders β-thalassemia and sickle cell anemia (SCA) can only be cured by allogeneic hematopoietic stem cell transplantation (HSCT). HSCT treatment of thalassemia has substantially improved over the last two decades, with advancements in preventive strategies, control of transplant-related complications, and preparative regimens. A risk class-based transplant...

Activated polymorphonuclear neutrophils play an important role in the pathogenesis of vaso-occlusive painful sickle cell crisis. Upon activation, polymorphonuclear neutrophils can form neutrophil extracellular traps. Neutrophil extracellular traps consist of a meshwork of extracellular DNA, nucleosomes, histones and neutrophil proteases. Neutrophil extracellular traps have been demonstrated to ...

Human hemoglobin messenger RNA was isolated by sucrose gradient centrifugation from reticulocytes of patients having various hemolytic anemias. Using a messenger RNA-dependent cell-free system derived entirely from rabbit reticulocytes, the human hemoglobin messenger RNA has been translated and the products analyzed by carboxymethylcellulose column chromatography. Normal messenger RNA directs s...

Pregnancy poses a unique challenge to patients with sickle cell disease and β-thalassemia, who often have exacerbations of hemolysis or anemia during the gestational period, experience higher rates of obstetric and fetal complications, and may have distinct underlying comorbidities related to vasculopathy and iron overload that can endanger maternal health. Optimal management of pregnant women ...