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Disorders of Erythrocytes

Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
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Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
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The hemoglobin E thalassemias.

Suthat Fucharoen1, David J Weatherall

  • 1Thalassemia Research Centre, Institute of Science and Technology for Research and Development, Mahidol University, Bangkok, Thailand. grsfc@mahidol.ac.th

Cold Spring Harbor Perspectives in Medicine
|August 22, 2012
PubMed
Summary
This summary is machine-generated.

Hemoglobin E (HbE) is a common variant causing mild beta thalassemia. Its combination with beta thalassemia leads to severe forms, common in Asia.

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Area of Science:

  • Hematology
  • Genetics

Background:

  • Hemoglobin E (HbE) is a prevalent structural hemoglobin variant in Asia.
  • HbE is a beta-hemoglobin variant with reduced production, exhibiting a mild beta-thalassemia phenotype.

Purpose of the Study:

  • To investigate the clinical significance of Hemoglobin E.
  • To understand HbE's interaction with alpha and beta thalassemia.

Main Methods:

  • Literature review on Hemoglobin E and thalassemia.
  • Analysis of clinical data on HbE interactions.

Main Results:

  • HbE interactions with alpha thalassemia cause diverse clinical disorders.
  • HbE/beta-thalassemia is the most common severe beta-thalassemia in Asia, accounting for 50% globally.

Conclusions:

  • Hemoglobin E is a significant factor in beta-thalassemia severity.
  • HbE/beta-thalassemia represents a major global health concern for severe thalassemia.