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Immune System: Sickle Cell Disease

Anemia is a blood disorder where the number of red blood cells is below normal. Red blood cells carry oxygen throughout the body. Anemia can be caused by the body destroying or losing too many red blood cells or not producing enough. Common causes include iron deficiency, infections, cancer, and inherited disorders. Inherited disorders like sickle cell disease, thalassemia, G6PD deficiency, and hereditary spherocytosis can lead to anemia by causing red blood cells to be destroyed faster than they can be replaced. Sickle cell disease causes red blood cells to take on a sickle shape and clog blood vessels. Thalassemia and G6PD deficiency impact hemoglobin production and protection of red blood

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50 views2 pages

Immune System: Sickle Cell Disease

Anemia is a blood disorder where the number of red blood cells is below normal. Red blood cells carry oxygen throughout the body. Anemia can be caused by the body destroying or losing too many red blood cells or not producing enough. Common causes include iron deficiency, infections, cancer, and inherited disorders. Inherited disorders like sickle cell disease, thalassemia, G6PD deficiency, and hereditary spherocytosis can lead to anemia by causing red blood cells to be destroyed faster than they can be replaced. Sickle cell disease causes red blood cells to take on a sickle shape and clog blood vessels. Thalassemia and G6PD deficiency impact hemoglobin production and protection of red blood

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tom
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Anemia is when the level of healthy red blood cells (RBCs) in the body becomes

too low. This can lead to health problems because RBCs contain hemoglobin,
which carries oxygen to the body's tissues. Anemia can cause a variety of
complications, including fatigue (tiredness) and stress on the body's organs.

Having fewer red blood cells than normal can happen because:

1. The body is destroying red blood cells.


2. Red blood cells are being lost.
3. The body is producing red blood cells too slowly.

Anemia a fairly common blood disorder with many causes. They include inherited
disorders, nutritional problems (such as an iron or vitamin deficiency), infections,
some kinds of cancer, and exposure to a drug or toxin.

Anemia From Destruction of Red Blood Cells


When the body destroys red blood cells (a process called hemolysis) more quickly
than normal, the bone marrow makes up for it by increasing production of new red
cells. But if red blood cells are destroyed faster than they can be replaced, a person
will develop anemia. This is called hemolytic anemia.
Autoimmune hemolytic anemia happens when the body's immune
system mistakenly attacks and destroys RBCs. This can happen because of disease,
but sometimes no cause is found.
Inherited hemolytic anemia is due to an inherited (passed down from parents to
their children) defect in the red blood cells. These defects can cause anemia.
Common forms of inherited hemolytic anemia include sickle cell
disease, thalassemia, glucose-6-phosphate dehydrogenase (G6PD) deficiency,
and hereditary spherocytosis.
 Sickle cell disease is a severe form of anemia that's most common in people of
African heritage (about 1 out of every 500 African-American children is born with
it). It affects hemoglobin, a protein in red blood cells that helps carry oxygen
throughout the body. RBCs with normal hemoglobin move easily through the
bloodstream because of their rounded shape and flexibility. Sickle cell disease
makes RBCs sticky, stiff, and more fragile, and they form into a curved, sickle
shape. Instead of moving through the bloodstream easily, sickle cells can clog
blood vessels. When blood can't get where it's needed, the body's tissues and
organs don't have the oxygen they need to stay healthy.
 Thalassemia affects the way the body makes hemoglobin. The body contains more
red blood cells than any other type of cell, and each has a life span of about 4
months. Each day, the body produces new RBCs to replace those that die or are
lost from the body. With thalassemia, the cells are destroyed at a faster rate,
leading to anemia.

Thalassemia major, also called Cooley's anemia is a severe form of anemia where
red blood cells are rapidly destroyed and iron is deposited in the vital
organs. Thalassemia minor causes less severe anemia.
 G6PD deficiency. G6PD is one of many enzymes that help the body process
carbohydrates and turn them into energy. G6PD also protects RBCs from harmful
byproducts that can gather when a person takes certain medicines or when the body
is fighting an infection. In people with G6PD deficiency, either the red blood cells
don't make enough G6PD or what's produced doesn't work correctly. Without
enough G6PD to protect them, the cells can be damaged or destroyed.

G6PD deficiency most commonly affects males of African heritage, although it has
been found in many other people.
 Hereditary spherocytosis is an inherited condition where red blood cells aren't
shaped correctly (like tiny spheres, instead of disks) and are very fragile because of
a genetic problem with a protein in the structure of the red blood cell. This causes
the cells to be easily destroyed and can cause anemia, jaundice (yellow-tinged
skin), and enlargement of the spleen. A family history increases the risk for this
disorder, which is most common in people of northern European heritage but can
affect all races.

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