Senegal haplotype is associated with higher HbF than benin and cameroon haplotypes in African children with sickle cell anemia

  title={Senegal haplotype is associated with higher HbF than benin and cameroon haplotypes in African children with sickle cell anemia},
  author={N. Green and M. Fabry and L. Kaptue-Noche and R. Nagel},
  journal={American Journal of Hematology},
Emerging Science of Hydroxyurea Therapy for Pediatric Sickle Cell Disease
Hydroxyurea (HU) is the sole approved pharmacological therapy for sickle cell disease (SCD). Higher levels of fetal hemoglobin (HbF) diminish deoxygenated sickle globin polymerization in vitro andExpand
Genótipos da talassemia alfa e haplótipos do agrupamento de genes da globina beta como moduladores de gravidade na Doença falciforme em crianças do Programa Estadual de Triagem Neonatal de Minas Gerais matriculados no Hmeocentro de Belo Horizonte da Fundação Hemominas
A presenca de -Tal associou-se significativamente com a diminuicao dos niveis de Hb total, Hb F, VCM, HCM, leucocitos e reticulocitOS, e nao influenciou os nivel de plaquetas. Expand
A Critical Interpretation on Sickle Cell Anaemia: A Genetic Disorder A Brief Review Study
HbS is a homozygous type of Sickle Cell Anaemia (HbSS). This is the product of a single point substitution of β-globin chain valine for glutamine 6. This limits the solubility of the red cells, whichExpand
Association between BCL11A, HSB1L-MYB, and XmnI γG-158 (C/T) gene polymorphism and hemoglobin F level in Egyptian sickle cell disease patients
Investigating HbF-enhancer haplotypes at BCL11A, HSB1L, and Xmn1 genetic polymorphisms to obtain a first overview of the genetic situation of SCD patients in Egypt and its impact on the severity of the disease found them to have no positive impact on baselineHbF levels solely but had if coexisted. Expand
Association of fetal hemoglobin level with frequency of acute pain episodes in sickle cell disease (HbS-only phenotype) patients.
Higher concentrations of HbF are associated with protection against frequent episodes of acute pain crisis in SCD patients, and were observed only in the 11-20 age group of cases in comparison to controls. Expand
Immunogenetics in sickle cell disease
The role of inflammatory genes encoding Toll-like receptors (TLR) in the occurrence of bacterial infections in patients with SCD was assessed, because infection is a leading cause of mortality in SCD, and TLR recognize a wide range of bacteria. Expand
A study on the genotype frequency of $$-158~\hbox {G}\gamma $$-158Gγ ($$\hbox {C}{\rightarrow }\hbox {T}$$C→T) Xmn1 polymorphism in a sickle cell trait cohort from Siwa Oasis, Egypt
In the absence of any homozygous genotype for the mutant allele (TT) in the studied cohort, any reasonable conclusion on the effect of polymorphism on increase in HbF could not be established. Expand
A study on the genotype frequency of -158 Gγ (C→T) Xmn1 polymorphism in a sickle cell trait cohort from Siwa Oasis, Egypt.
Studying genotype frequency of the Xmn1 γG globin polymorphism (-158C>T ) in Siwa Oasis, Egypt can be considered as a starting point for further research targeting this community sector and any association with increased HbF expression could not be established. Expand
Fetal haemoglobin induction in sickle cell disease
Autologous transplant of edited haematopoietic stem cells holds promise as a cure for SCD, either through HbF induction or correction of the causative mutation, but several technical and safety hurdles must be overcome before this therapy can be offered widely, and pharmacological therapies are still needed. Expand


The Senegal DNA haplotype is associated with the amelioration of anemia in African-American sickle cell anemia patients.
It is concluded that the Senegal beta-like globin gene cluster haplotype is associated with an amelioration of the hemolytic anemia that characterizes sickle cell disease. Expand
β-Cluster Haplotypes, α-Gene Status, and Hematological Data from SS, SC, and S-β-Thalassemia Patients in Southern California
The variable levels of hemoglobin, MCV, Hb F, G gamma chains, and Hb A2 are in response to the heterogeneous genetic mix of the beta S-gene-cluster haplotypes and alpha-thalassemia-2 in American patients with sickle cell anemia. Expand
Hematologically and genetically distinct forms of sickle cell anemia in Africa. The Senegal type and the Benin type.
The differences in the hematologic characteristics of patients with sickle cell anemia from the first two geographical areas are studied to mean that the gamma-chain composition and the hemoglobin F level are haplotype linked and that the decrease in the percentage of dense cells and irreversibly sickled cells is secondary to the elevation in the hemochemistry F level. Expand
Dense cells in sickle cell anemia: the effects of gene interaction.
The results indicate that it is necessary to consider sickle cell anemia not only as a single gene defect, but also as a disease whose clinical expression is the result of a group of genes capable of interacting at the phenotypic level. Expand
Some aspects of the pathophysiology of homozygous Hb CC erythrocytes.
The contradiction was resolved by rheological studies on isolated rat mesoappendix preparations and erythrocyte diameter measurements that lead to the conclusion that the hemorheological properties of CC cells in the microcirculation are nearly normal because their increased viscosity is offset by their smaller diameter and size. Expand
Functional hyposplenia contributes to overwhelming infection in SS disease and may be assessed by Howell-Jolly bodies and ≥ scans and useful in determining a strategy for prophylaxis against severe bacterial infections. Expand
Postnatal Changes in the Chemical Heterogeneity of Human Fetal Hemoglobin
The change in the ratio of production of structurally different γ chains as a function of postnatal age indicates a rather complex mechanism which probably involves an unequal repression of the γ chain structural genes. Expand