Related Experiment Video
Updated: May 27, 2026
05:23
Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Iron depletion: an ameliorating factor for sickle cell disease?
P C Giordano1, W Huisman, C L Harteveld
1Hemoglobinopathies Laboratory, Department of Human and Clinical Genetics, Leiden University Medical Center, Einthovenweg 20, 2300RC Leiden, The Netherlands.
ISRN Hematology
|November 16, 2011
Summary
Iron depletion may benefit sickle cell disease (SCD) patients by reducing HbS and increasing HbF, potentially lessening disease severity. Further studies and iron chelation therapy are recommended for managing iron overload in SCD.
Area of Science:
- Hematology
- Molecular Biology
- Medical Practice
Background:
- Sickle cell disease (SCD) is a genetic blood disorder characterized by abnormal hemoglobin (HbS).
- Managing SCD involves addressing complications arising from the disease, including iron overload from transfusions.
- Current treatments aim to alleviate symptoms and prevent complications.
Observation:
- Two case studies observed a potential link between iron depletion and beneficial effects in SCD patients.
- Iron depletion appeared to decrease the formation of sickle hemoglobin (HbS).
- Simultaneously, iron depletion was associated with an increase in fetal hemoglobin (HbF) levels.
Findings:
- The observed reduction in HbS and increase in HbF suggests a possible mechanism for mitigating SCD severity.
- Iron depletion may favorably alter hemoglobin composition in SCD patients.
- These findings highlight the potential role of iron modulation in SCD management.
Implications:
- Further clinical studies comparing SCD patients with identical genotypes, with and without iron depletion, are warranted.
- Monitoring disease progression under controlled iron levels could validate these observations.
- Chelation therapy should be considered to manage iron overload in SCD patients, potentially improving outcomes.
Related Concept Videos
01:27Disorders 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...
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...
01:24Factors Affecting Erythropoiesis
The cardiovascular system regulates the number of erythrocytes in the bloodstream to ensure optimal oxygen transport. It also prevents over-proliferation of these cells, which helps to maintain blood viscosity and flow rate.
Several factors influence the erythrocyte production rate, with tissue oxygen level being among the most critical. Intense exercise or high altitudes can cause tissue hypoxia, which triggers the kidneys to release more erythropoietin (EPO) into the bloodstream.
EPO then...
Several factors influence the erythrocyte production rate, with tissue oxygen level being among the most critical. Intense exercise or high altitudes can cause tissue hypoxia, which triggers the kidneys to release more erythropoietin (EPO) into the bloodstream.
EPO then...
00:57The Periodic Table and Organismal Elements
OverviewElements are the smallest units of matter that cannot be broken down further by chemical processes. There are 118 known elements, but not all of these are naturally-occurring, and fewer still are essential for life. Living matter is composed primarily of carbon, nitrogen, hydrogen, and oxygen, with smaller amounts of other elements like calcium, phosphorus, potassium, and sulfur. Other elements are also necessary for life but only in trace amounts.The Periodic Table Provides Information...
01:22Bone Marrow Sampling and Transplants
Bone marrow transplant is a potential cure for several diseases, including cancer and specific genetic disorders. Notably, this procedure is applicable for patients suffering from aplastic anemia, certain types of leukemia, severe combined immunodeficiency disease (SCID), Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma, thalassemia, sickle-cell disease, and certain cancers.
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy the...
The transplant begins with high doses of chemotherapy and radiation treatment, which aim to destroy the...
01:49Multiple Allele Traits
The Concept of Multiple Allelism
01:14Erythropoiesis
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...