Aplastic Anemia

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APLASTIC ANEMIA

 A condition that occurs when the body stops producing enough new blood cells.
 A rare and serious condition
 Can develop at any age.
 It can occur suddenly, or it can come on slowly and worsen over time.
 It can be mild or severe.
 The condition causes easy fatigability and more prone to infections and uncontrolled bleeding.
Treatment for aplastic anemia might include medications, blood transfusions or a stem cell transplant, also known as a
bone marrow transplant.

Symptoms

Aplastic anemia can have no symptoms.


When present, signs and symptoms can include:
o Fatigue
o Shortness of breath
o Rapid or irregular heart rate
o Pale skin
o Frequent or prolonged infections
o Unexplained or easy bruising
o Nosebleeds and bleeding gums
o Prolonged bleeding from cuts
o Skin rash
o Dizziness
o Headache
o Fever
Aplastic anemia can be short-lived, or it can become chronic. It can be severe and even fatal.

CAUSES
Stem cells in the bone marrow produce blood cells — red cells, white cells and platelets.
In aplastic anemia, stem cells are damaged.
As a result, the bone marrow is either empty (aplastic) or contains few blood cells (hypoplastic).
The most common cause of aplastic anemia: the immune system attacks the stem cells in the bone marrow.
Other factors that can injure bone marrow and affect blood cell production include:

 Radiation and chemotherapy treatments. While these cancer-fighting therapies kill cancer cells, they can also
damage healthy cells, including stem cells in bone marrow. Aplastic anemia can be a temporary side effect of
these treatments.

 Exposure to toxic chemicals. Toxic chemicals, such as some used in pesticides and insecticides, and benzene, an
ingredient in gasoline, have been linked to aplastic anemia. This type of anemia might improve if repeated
exposure to the chemicals that caused the illness is avoided.

 Use of certain drugs. Some medications, such as those used to treat rheumatoid arthritis and some antibiotics,
can cause aplastic anemia.

 Autoimmune disorders. An autoimmune disorder, in which the immune system attacks healthy cells, might
involve stem cells in the bone marrow.

 A viral infection. Viral infections that affect bone marrow can play a role in the development of aplastic anemia.
Viruses that have been linked to aplastic anemia include hepatitis, Epstein-Barr, cytomegalovirus, parvovirus B19
and HIV.

 Pregnancy. The immune system might attack the bone marrow during pregnancy.

 Unknown factors. In many cases, doctors aren't able to identify the cause of aplastic anemia (idiopathic aplastic
anemia).

Connections with other rare disorders

Some people with aplastic anemia also have a rare disorder known as paroxysmal nocturnal hemoglobinuria,
which causes red blood cells to break down too soon. This condition can lead to aplastic anemia, or aplastic anemia can
evolve into paroxysmal nocturnal hemoglobinuria.

Fanconi's anemia is a rare, inherited disease that leads to aplastic anemia. Children born with it tend to be
smaller than average and have birth defects, such as underdeveloped limbs. The disease is diagnosed with the help of
blood tests.

Risk factors

Aplastic anemia is rare. Factors that can increase risk include:


 Treatment with high-dose radiation or chemotherapy for cancer
 Exposure to toxic chemicals
 The use of some prescription drugs — such as chloramphenicol, which is used to treat bacterial infections, and
gold compounds used to treat rheumatoid arthritis
 Certain blood diseases, autoimmune disorders and serious infections
 Pregnancy, rarely
Diagnosis
 Many tests are used to diagnose aplastic anemia.
 Confirm a diagnosis of aplastic anemia, look for its cause, and find out how severe it is
 Rule out other conditions that may cause similar symptoms
 Check for paroxysmal nocturnal hemoglobinuria (PNH)
 The following tests can help diagnose aplastic anemia:
BLOOD TESTS:

Complete Blood Count (CBC)


o The first test used to diagnose aplastic anemia
o Measures hemoglobin and hematocrit levels.
o A low level of both is a sign of anemia.
o The normal range of these levels varies in certain racial and ethnic populations.
o Red blood cells, white blood cells and platelets count.
o Abnormal results may be a sign of aplastic anemia, an infection, or another condition.
o In aplastic anemia, red blood cell, white blood cell and platelet levels are low.
o Mean corpuscular volume (MCV).
o MCV is a measure of the average size of your red blood cells.
o The results may be a clue as to the cause of the anemia.

Reticulocyte Count

 Measures the number of young red blood cells in the blood.


 The test shows whether the bone marrow is making red blood cells at the correct rate.
 In aplastic anemia, have low reticulocyte levels.

Bone marrow tests 


 Show whether the bone marrow is healthy and making enough blood cells.
 The two bone marrow tests are aspiration and biopsy.

Bone marrow biopsy


Confirmatory diagnosis of aplastic anemia. DO other tests to determine the cause of aplastic anemia.
 A bone marrow biopsy may be done at the same time as an aspiration or afterward.
o With the use of a needle, remove a small amount of bone marrow tissue from a large bone such as the hipbone.
o The tissue is examined under a microscope to check the number and types of cells in the bone marrow and rule
out other blood-related diseases.
o In aplastic anemia, bone marrow contains fewer than normal number of all three types of blood cells.
Bone marrow aspiration
 To determine why the bone marrow isn't making enough blood cells.
 Removes a small amount of bone marrow fluid through a needle.
 The sample is checked microscopically to look for faulty cells.
Other Tests

Other tests may be needed to rule out other conditions that can cause symptoms similar to those of aplastic anemia.
These tests may include:
 X ray, computed tomography (CT) scan, or an ultrasound imaging test.
 These tests can show enlarged lymph nodes in the abdomen.
 Enlarged lymph nodes may be a sign of blood cancer.
 May use these tests to visualize the kidneys and the bones in the arms and hands, which are sometimes
abnormal in young people who have Fanconi anemia. This type of anemia can lead to aplastic anemia.
Chest x ray
 Visualize structures such as the heart, lungs, and blood vessels.
 A chest x ray may be used to rule out infections.
Liver tests and viral studies
 These tests are used to check for liver diseases and viruses.

Vitamin B12 and folate levels in the blood


 These tests can help rule out anemia caused by vitamin deficiency.

Recommend blood tests for PNH and to check the immune system for antibodies.
o Antibodies in the immune system may attack the bone marrow cells that may cause aplastic anemia.

Treatment

Will depend on the severity of the condition and age


Treatment might include observation, blood transfusions, medications, or bone marrow transplantation. Severe aplastic
anemia, in which the blood cell counts are extremely low, is life-threatening and requires immediate hospitalization.

Blood transfusions

Although not a cure for aplastic anemia, blood transfusions can control bleeding and relieve symptoms by providing
blood cells the bone marrow isn't producing.

 Red blood cells. It raises red blood cell counts and helps relieve anemia and fatigue.

 Platelets. To help prevent excessive bleeding.

While there's generally no limit to the number of blood transfusions, complications can sometimes arise with multiple
transfusions.

Transfused red blood cells contain iron that can accumulate in the body and can damage vital organs if an iron overload
isn't treated.
Medications can help rid the body of excess iron.

Over time the body can develop antibodies to transfused blood cells, making them less effective at relieving symptoms.
The use of immunosuppressant medication makes this complication less likely.

Stem cell transplant

A stem cell transplant to rebuild the bone marrow with stem cells from a donor might be the only successful treatment
option for people with severe aplastic anemia. A stem cell transplant, also called a bone marrow transplant, is generally
the treatment of choice for people who are younger and have a matching donor — most often a sibling.

If a donor is found, the diseased bone marrow is first depleted with radiation or chemotherapy. Healthy stem cells from
the donor are filtered from the blood. The healthy stem cells are injected intravenously into the bloodstream, where
they migrate to the bone marrow cavities and begin creating new blood cells.

The procedure requires a lengthy hospital stay. After the transplant, the patient receives drugs to help prevent rejection
of the donated stem cells.

A stem cell transplant carries risks. The body may reject the transplant, leading to life-threatening complications. In
addition, not everyone is a candidate for transplantation or can find a suitable donor.

Immunosuppressants

For people who can't undergo a bone marrow transplant or for those whose aplastic anemia is due to an autoimmune
disorder, treatment can involve drugs that alter or suppress the immune system (immunosuppressants).

Drugs such as cyclosporine (Gengraf, Neoral, Sandimmune) and anti-thymocyte globulin suppress the activity of immune
cells that are damaging the bone marrow. This helps the bone marrow recover and generate new blood cells.
Cyclosporine and anti-thymocyte globulin are often used together.

Corticosteroids, such as methylprednisolone (Medrol, Solu-Medrol), are often used with these drugs.

Although effective, these drugs further weaken the immune system. It's also possible for anemia to return after the
patient stops taking these drugs.

Bone marrow stimulants

Certain drugs — including colony-stimulating factors, such as sargramostim (Leukine), filgrastim (Neupogen) and
pegfilgrastim (Neulasta), epoetin alfa (Epogen/Procrit), and eltrombopag (Promacta) — help stimulate the bone marrow
to produce new blood cells. Growth factors are often used with immune-suppressing drugs.
Antibiotics, antivirals
Having aplastic anemia weakens the immune system, which leaves the patient more prone to infections.

Other treatments

Aplastic anemia caused by radiation and chemotherapy treatments for cancer usually improves after those treatments
stop. The same is true for most other drugs that induce aplastic anemia.

Pregnant women with aplastic anemia are treated with blood transfusions. For many women, pregnancy-related aplastic
anemia improves once the pregnancy ends. If that doesn't happen, treatment is still necessary.

Prevention

There's no prevention for most cases of aplastic anemia.


Avoiding exposure to insecticides, herbicides, organic solvents, paint removers and other toxic chemicals might lower
the risk of the disease.

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