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Related Concept Videos

Hemoglobin01:24

Hemoglobin

Hemoglobin is a globular protein made up of four subunits. Two of these subunits are alpha chains, and the other two are beta chains. Each subunit contains a molecule of heme, which has an iron atom and can bind to oxygen. When an oxygen molecule binds to one heme group, it changes the shape of hemoglobin, making it easier for the other heme groups to bind oxygen as well.
When all four heme groups are bound to oxygen, the resulting molecule is called oxyhemoglobin. As a result, arterial blood...
Disorders of Erythrocytes01:27

Disorders of Erythrocytes

Disorders of erythrocytes, or red blood cells (RBCs), include a range of conditions affecting their number, shape, or function.
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
Multiple Allele Traits01:49

Multiple Allele Traits

The Concept of Multiple Allelism
Oxygen Transport in the Blood01:27

Oxygen Transport in the Blood

Hemoglobin (Hb) is a crucial molecule in the human body, consisting of four polypeptide chains, each bound to an iron-containing heme group. This unique structure enables hemoglobin to bind to oxygen, with each molecule capable of combining with four molecules of oxygen, leading to rapid and reversible oxygen loading. When fully loaded with oxygen, it is called oxyhemoglobin, while hemoglobin that has released oxygen is called reduced hemoglobin or deoxyhemoglobin. As hemoglobin binds oxygen,...
Erythropoiesis01:14

Erythropoiesis

Red blood cells  (RBCs) transport oxygen to all body tissues. These cells survive only for 120 days and then need to be replenished. Erythropoiesis is the process of RBC production. In healthy individuals, erythropoiesis ensures all tissues are amply supplied with oxygen. In addition, blood loss due to injury leads to a drop in the physiological oxygen level that will cause erythropoiesis. Any defect in erythropoiesis leads to several physiological disorders, including thalassemia, anemia, and...
Erythropoiesis01:14

Erythropoiesis

Red blood cells  (RBCs) transport oxygen to all body tissues. These cells survive only for 120 days and then need to be replenished. Erythropoiesis is the process of RBC production. In healthy individuals, erythropoiesis ensures all tissues are amply supplied with oxygen. In addition, blood loss due to injury leads to a drop in the physiological oxygen level that will cause erythropoiesis. Any defect in erythropoiesis leads to several physiological disorders, including thalassemia, anemia, and...

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Related Experiment Video

Updated: Jul 6, 2026

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
11:59

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies

Published on: September 6, 2017

Studies in haemoglobin E beta-thalassaemia.

Nancy F Olivieri1, Giulia M Muraca, Angela O'Donnell

  • 1Hemoglobinopathy Research Program, Toronto General Hospital, University of Toronto, Toronto, ON, Canada. noliv@attglobal.net

British Journal of Haematology
|April 16, 2008
PubMed
Summary
This summary is machine-generated.

Haemoglobin E beta-thalassaemia, common in Asia, shows diverse clinical outcomes due to genetic and environmental factors. Many patients can be managed without transfusions, even with low hemoglobin levels, suggesting cost-effective approaches.

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Measurement of Heme Synthesis Levels in Mammalian Cells
09:43

Measurement of Heme Synthesis Levels in Mammalian Cells

Published on: July 9, 2015

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Last Updated: Jul 6, 2026

Detection of Residual Donor Erythroid Progenitor Cells after Hematopoietic Stem Cell Transplantation for Patients with Hemoglobinopathies
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Published on: September 6, 2017

Measurement of Heme Synthesis Levels in Mammalian Cells
09:43

Measurement of Heme Synthesis Levels in Mammalian Cells

Published on: July 9, 2015

Area of Science:

  • Hematology
  • Genetics
  • Clinical Medicine

Background:

  • Haemoglobin E beta-thalassaemia is a prevalent severe thalassaemia in Asia.
  • Its natural history, clinical diversity, and optimal management remain poorly understood.
  • Current management often relies on demand transfusions in a haphazard manner.

Purpose of the Study:

  • To investigate the natural history and phenotypic diversity of Haemoglobin E beta-thalassaemia.
  • To identify genetic and environmental factors influencing disease presentation.
  • To explore alternative management strategies beyond transfusion.

Main Methods:

  • A 5-year cohort study of Sri Lankan patients with Haemoglobin E beta-thalassaemia.
  • Analysis of genetic modifiers affecting haemoglobin F production.
  • Assessment of environmental factors like malaria and age-related anaemia adaptation.

Main Results:

  • Several genetic and environmental factors were identified as contributors to phenotypic diversity.
  • Modifiers of haemoglobin F production, malaria exposure, and age-related adaptation to anaemia were implicated.
  • Low haemoglobin levels did not necessitate transfusion in many patients.

Conclusions:

  • Haemoglobin E beta-thalassaemia exhibits significant clinical variability.
  • Management can often avoid transfusions, even with low haemoglobin.
  • Age-related adaptation suggests opportunities for more cost-effective management strategies.