Congenital Hemolytic Anemia accelerated destruction of red blood cells due to an inherited

defect, such as in the membrane in hereditary spherocytosis.

What is Hemolytic Anemia?
Your bone marrow is responsible for producing red blood cells, white blood cells, and
platelets (cells responsible for clotting). Hemolytic anemia is a condition that involves only
the red blood cells. When old red blood cells die, the bone marrow produces new ones to
maintain balance. Hemolytic anemia occurs when red blood cells die sooner than the bone
marrow can produce them. The scientific term for red blood cell destruction ishemolysis.
There are two forms of hemolytic anemia: intrinsic and extrinsic.
Extrinsic hemolytic anemia develops when the spleen traps and destroys healthy red
blood cells. It can also come from red blood cell destruction due to infection, tumors,
autoimmune disorders, medication side effects, leukemia, or lymphoma. Intrinsic
hemolytic anemia develops when the red blood cells produced by the body are defective.
The condition is often inherited, such as in people with sickle cell anemia or thalassemia.
Anyone of any age can develop hemolytic anemia. However, according to the National
Heart, Lung, and Blood Institute, hemolytic anemia seems to affect more African-Americans
than Caucasians (NHLBI). This is likely because sickle cell anemia is more prevalent amongst
African-Americans.
Part 2 of 5: Causes
Causes of Hemolytic Anemia
The cause of hemolytic anemia is red blood cell destruction. Red blood cell destruction can
arise from blood disorders, toxins, or infection.

Among others, underlying causes of of hemolytic anemia include:

hepatitis

Epstein-Barr Virus (EBV)

typhoid fever

sickle cell anemia

E. coli

streptococcus

leukemia

lymphoma

tumors

penicillin

pain medication

acetaminophen

lupus

Wiscott Aldridge syndrome

Some cases of anemia have no known cause.

Part 3 of 5: Symptoms
What are the Symptoms of Hemolytic Anemia?
Some symptoms of hemolytic anemia are the same as other forms of anemia.

These common symptoms include:

paleness of the skin

fatigue

fever

confusion

lightheadedness

dizziness

weakness/inability to do physical activity

Other, less common signs and symptoms seen in patients with hemolytic anemia include:

dark urine

yellowing of the skin and the whites of the eyes (jaundice)

heart murmur

increased heart rate

enlarged spleen
enlarged liver
Symptoms
Symptoms vary widely. Mild cases may not cause any symptoms. The condition may only be
discovered during a routine blood test.
In other people, obvious symptoms of anemia can develop. These include:

Pale skin

Fatigue

Weakness

Dizziness

Lightheadedness

Breathlessness

Yellowing of the skin and eyes

Dark tea-colored urine

Expected Duration
How long hemolytic anemia lasts depends on its cause.
Hemolytic anemia caused by a medication or infection is usually temporary. It goes away
when the drug is discontinued or the infection is treated.
Hemolytic anemia caused by an inherited illness is a lifelong condition. The impact on a
person's quality of life and life span can vary greatly. It depends on the specific inherited
disorder and its severity. Some people don't have any symptoms. Others have severe,
persistent symptoms.
Prevention
Hemolytic anemia caused by medication can be prevented. Avoid the drug that is causing
the problem.
There is no way to prevent inherited hemolytic anemia. If an inherited anemia runs in your
family, you may undergo genetic testing. This can assess your chances of passing it on to
your children.
Treatment
Treatment for hemolytic anemia depends on its cause and severity.

Inherited abnormalities in red cell membranes If hemoglobin levels are

adequate, you may not need treatment. If hemoglobin levels drop very low, a blood
transfusion may be needed. In rare cases, the spleen is removed.

Inherited enzyme deficiencies inside red cells Blood transfusions are

necessary to treat severe symptoms.

Hemoglobin disorders Treatment is not necessary if the illness is mild or

does not cause any symptoms.

People with more severe forms of thalassemia require repeated blood transfusions. Some
may be candidates for a bone marrow transplant.
Sickle cell anemia may be treated with:

folic acid supplements

blood transfusions

antibiotics

a drug called hydroxyurea (Hydrea)

Physical damage to red blood cells This may be treated with folic acid and
iron supplements. Blood transfusions may be necessary.

An autoimmune response Treatment depends on the cause and severity. If a

drug or infection is causing the anemia, it may be enough to stop the drug or recover
from the infection.
For more severe cases, treatment may include:

corticosteroid medication

intravenous immune globulin infusions

immunosuppressive drugs

removal of the spleen

Hypersplenism For severe symptoms, the spleen is removed.

People with severe, chronic hemolytic anemia, especially those with sickle cell anemia or
thalassemia, may require multiple blood transfusions. The iron in hemoglobin can
accumulate in the body, causing iron overload and organ damage. Drugs called iron
chelators, which bind the iron and enhance the body's removal of iron, help prevent organ
damage from iron overload.

When to Call A Doctor

Call your doctor promptly if you develop symptoms of anemia. Also call if you notice a
yellowish tint in your skin or in the whites of your eyes.
If an inherited form of anemia runs in your family, you may wish to consider genetic testing
before you start a family.

How Is Hemolytic Anemia Treated?

Treatment options for hemolytic anemia differ depending on severity of the condition, your
age, your health, and your tolerance to certain medications.
Treatment options for hemolytic anemia include:

blood transfusion

intravenous immune globulin

corticosteroid medication

surgery
Blood Transfusion
A blood transfusion is given to quickly increase your red blood cell count and to replace
destroyed red blood cells with new ones.
Intravenous Immune Globulin
A low blood cell count can negatively affect the way your immune system fights infection.
You may be given immune globulin through an IV in the hospital to improve your immune
system function.
Corticosteroids
In the case of an extrinsic form of hemolytic anemia of autoimmune origin, corticosteroids
are used to stop your immune system from making antibodies that destroy red blood cells.

Surgery
In severe cases, your spleen may need to be removed.
Special Considerations
Some people have hemolytic anemia thats reactive to cold. If you have this condition, stay
warm as much as possible to reduce symptoms.
Inherited forms of hemolytic anemia are addressed differently than acute (short-term) forms
of the condition. Your doctor may approach treatment differently if you have an inherited
form of hemolytic anemia.
Prognosis
The outlook for hemolytic anemia depends on its cause and severity. The underlying health
of the affected person also affects the prognosis.
Cases that are caused by medications or infections usually go away quickly.
People with autoimmune hemolytic anemia usually respond well to treatment.
The outlook for people with inherited hemolytic anemias depends on the type of inherited
illness and its severity.