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={Nancy S. Green and Mary E. Fabry and L Kaptue-Noche and Ronald L. 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 and

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.

Hematological and molecular analyses of the HbS allele among the Sudanese population

As a result of sickle cell anemia, this investigation found changes in hematological parameters in the Sudanese population, and a new haplotype of the S gene was discovered.

Hematological, Biochemical Properties, and Clinical Correlates of Hemoglobin S Variant Disorder: A New Insight Into Sickle Cell Trait

A schematic diagnostic paradigm for SCT was proposed that could help prevention of the Hb S variant disorder and proper management of carriers and might be applied in pre-marital screening, particularly in those with family history of H b S disorder.

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, which

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.

Evaluation of correlation between clinical profile and amount of abnormal hemoglobin in sickle cell hemoglobinopathy

Clinical profile of sickle cell anemia is observed to be moderate i.e. less severe in this region as compared to reported in African Countries, which point strongly to the recommendation of the therapy to raise the HbF level in patients wherever it is low.

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.



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.

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.


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.

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.

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.

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.

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.