What is Aplastic Anemia?

Aplastic Anemia is a rare disease which causes the body to fail to produce enough blood cells. Aplastic is used to refer to the abnormal functioning of the bone marrow while anemia refers to shortage of blood cells in the body. The disease reacts by attacking the bone marrow which is responsible for the production stem cells which in turn produce blood cells and other stem cells in the body. These include the red blood cells which the body uses to transport oxygen to body organs; white blood cells which are responsible for protecting the body from infections; and platelets which aid in blood clotting thus prevent excessive bleeding. While the body needs to make new blood cells everyday as older ones are destroyed, an infected bone marrow fails to provide adequately for this demand leading to weakness of the body and inability to fight diseases and infections. The occurrence of Aplastic Anemia may take months or weeks before it becomes evident although in other circumstances it could be sudden. Failure of treatment could lead to detrimental effects hence the reason why it should not be ignored.

The causes of Aplastic Anemia are hard to determine although doctors in most cases cite that the disease could be as a result of inherited disorders in 20 percent of the patients. High exposure to radiation, toxic materials such as chemicals and viruses may also cause Aplastic Anemia. This disease can be said to be an auto-immune disease because it attacks the bone marrow and disables stem cells so that the are unable to produce enough blood as required by the body. Once the immune system starts attacking the bone marrow leading to less supply of blood cells, the best solution is the use of immunosuppresants to slow down the system.

People with severe cases of Aplastic Anemia are exposed to high risk of infections due to the limited white blood cells required to keep infections at bay. A person suffering from Aplastic Anemia is likely to feel fatigued owing to the limited circulation of oxygen into the body. Most patients report feeling weak, falling short of breath, having constant headaches, easy bruising and irregular heartbeat. Nausea and constant fainting are also signs that one could be suffering from the disease. Due to the low levels of platelets in the body, patients may suffer severe bleeding whenever they get hurt. Platelets serve the purpose of enhancing clotting of blood so that bleeding is stopped. Shortage would therefore automatically reduce the body’s ability to stop bleeding normally.

Treatment for Aplastic Anemia

Once the diagnosis of Aplastic Anemia is done, patients have a variety of treatment options that they can opt to take to cure the disease. The treatment of Aplastic Anemia is dependent on how severe the case is. Severe cases could be dangerous hence the need for immediate treatment using the most effective treatment. Usually, once diagnosis of severe Aplastic Anemia is made, blood transfusion helps to contain the situation before a more suitable form of treatment can be decided on. There are various treatments that are available for the treatment of Aplastic Anemia in most modern hospitals with the most common ones being bone marrow transplant and immunosupressants. A more recent treatment known as the High-Dose Clyclophosphamide which uses chemotherapy to treat Aplastic Anemia is also available even though it is on clinical trial in most hospitals. Other forms of treatment are usually meant to contain the disease to prevent it from endangering the patient’s life. They include use of growth factors, bone marrow stimulants, antibiotics, anti-retrovirals and use of iron supplements.

Bone marrow transplant is the most commonly used type of treatment. The patient’s bone marrow is extracted and a matching donor’s bone marrow is then injected into the patient’s bones. The bone marrow may take around three to four weeks before it can start production of stem cells. Bone marrow therapy effectively solves severe cases of Aplastic Anemia if the patient’s body adapts well to the donated bone marrow. It however poses a challenge once the body reacts to the blood cells produced by donor’s bone marrow leading to serious effects. Finding a matching donor could also be challenging.

Immunosuppressive therapy is used where a disorder causes the immune system to attack blood cells which are produced by the bone marrow such that they become deficient. The treatment works by suppressing the immune system so that the attack on the blood cells is reduced. This type of treatment is normally used by older people who do not want to undergo chemotherapy and bone marrow transplants; patients who have reacted negatively to bone marrow transplants and those who cannot find a matching donor. The limitation of using this method of treatment is that it causes the reduction the normal body immunity leaving the patient at high risk of other diseases and infections.

High-Dose Cyclophosphamide uses chemotherapy to treat the occurrence of harmful lymphocytes that destroy blood cells produced by the bone marrow. The treatment is combined with a growth factor known as GCSF which helps to promote the development of blood cells by the bone marrow. This treatment eliminates the lymphocytes only leaving healthy stem cells which continue the production of the blood cells. High-Dose Cyclophosphamide is known to have a high relapse rate which is why patients have preferred to use it even though it is in clinical trial.

Neglis Aplastic Anemia

Neglis Aplastic Anemia is a type of disease which affects the bone marrow such that it cannot effectively replenish the blood cells that are required by the body. This is mostly caused by disorders which cause the bone marrow not to function normally. Neglis Aplastic Anemia is in most cases acquired which accounts for 80 percent of the diagnosis while 20 percent of the cases are inherited disorders. A disorder such as lupus which is a condition that causes the immune system to attack healthy cells is a common cause of Neglis Aplastic Anemia. The immune system takes to attacking the blood cells that are produced by the bone marrow such that there a shortage occurs in the body. This can however be treated using a method known as immunosupressive therapy which controls the activity of the immune system to reduce the attack on blood cells. Exposures to radioactivity such as during chemotherapy or in industries as well as exposure to harmful chemicals are well known causes of Neglis Aplastic Anemia. Apart from these, certain conditions such as pregnancy and HIV can cause temporary cases of Neglis Aplastic Anemia which usually disappears after a short time. When the body cannot replenish its supply of the blood cells, it becomes life-threatening because the normal body functions are likely to be curtailed.

A patient suffering from Neglis Aplastic Anemia is likely to feel exhausted which can be explained by the fact that oxygen is not flowing in the right amounts. This is due to the shortage of red blood cells which are responsible for oxygen supply in the body. Apart from exhaustion, one is likely to feel dizzy, weak and in extreme cases suffer from fainting sprees. This could be dangerous as one is likely to suffer serious injuries. Patients are also prone to infections, easy bruising and uncontrolled bleeding. The white blood cells need to be replaced from time to time in order to aid the body in fighting off infections but when they cannot be effectively replaced then the result is poor immunity. The other type of blood cells is the platelets which help in clotting of blood. These cells ensure that a person does not lose excessive blood when injury occurs by clotting blood and sealing the wound. The shortage of platelets in the body is likely to cause excessive loss of blood by patients whenever they get hurt.

Aplastic Anemia Symptoms

Aplastic anemia is a disorder of the bone marrow in the human body. Let it be known, however, that aplastic anemia does not just affect the human race, as there have been cases found in some species of ferret. In aplastic anemia, there is a very low count of all the blood cell groups; namely the white blood cells, the red blood cells, and the blood platelets, and the official medical term of this process is pancytopenia. A normal case of anemia is, in most cases, not much to worry about, and your family doctor will merely tell you to introduce more iron into your diet, and take vitamin B12 supplements. Anemia is merely a low count of red blood cells and can be easily managed. This is not the same with aplastic anemia, and medical assistance must be sought if you suspect that you have this disorder.

There are at least 4 aplastic anemia symptoms; these are anemia itself, where your blood is deficient of hemoglobin, and heart palpitations may accompany, although this can be a symptom of many other medical conditions. Pallor may also be present, which is the whitening effect of the skin; when this happens, many people will say that the person “has seen a ghost”. Other aplastic anemia symptoms include thrombocytopenia, which is the low count of platelets in the blood. This symptom cannot be accurately determined unless you have your cells counted on a regular basis, and this can only be done by a doctor through medical tests; it is also characterized by excessive bruising. Leukopenia is the next, which is the low count of the white blood cells in the body - this, like the thrombocytopenia, cannot be known without the proper medical test, however, it does increase the risk of the patient getting all sorts of infections. The last symptom is called reticulocytopenia, or Aplastic Crisis. This is the abnormal decrease of the body’s red blood cell count.

Many of the symptoms may occur at the same time, however, in rare cases, there is a chance that these symptoms do not show up until it is too late for doctors to effectively help the patient. This is why it is highly important for you to get to your family doctor for a monthly full body check up.

Aplastic Anemia Definition

According to the American Heritage Dictionary, their aplastic anemia definition is given as:

“A form of anemia in which the capacity of the bone marrow to generate red blood cells, is defective. This anemia may be caused by bone marrow disease or exposure to toxic agents, such as radiation, chemicals, or drugs.”

Aplastic, also called hypoplastic is a term meaning “empty” and is used to identify the specific anemia where the bones are literally empty of blood. There are different groups of people that develop aplastic anemia and generally about 2 million to 4 million people suffer from it each year. The three age groups affected the most are from 2-5 years old, from 20-25 years old, and between 55 and 60 years old. There are many causes of this disease that prevent the bone marrow from producing the correct amounts of red blood cells, white blood cells, and platelets.

The aplastic anemia definition was termed in 1904 after various studies into the concept had been made after a pregnant woman died from bone marrow failure in 1888. This rare disease has many known causes, but also many that are unknown to doctors even after the patient has been diagnosed. The only way to make an accurate diagnosis is to perform a biopsy of the bone marrow in which the following will be tested:

· B12 count (iron count in the blood)

· Serum folate

· Drug screen

· CBC (complete blood count) with platelets and a pap smear

· Viral serologies

These biopsy results will show the causes (in most cases) of the aplastic anemia as well as how advanced the disease is. The causes that are known include exposure to drugs or chemicals like benzene, pregnancy, radiation, destruction of stem cells due to infections or medication, autoimmune disorders, and some inherited cases. Looking for the symptoms of aplastic anemia you will look for excessive bruising and hemorrhaging which indicates a low platelet count, pale skin or malaise, indicating a low red blood cell count, and severe infections, due to a low white blood cell count. In the early stages of aplastic anemia, blood transfusions and various other treatments can be used, but in severe cases a bone marrow transplant is necessary. This is a dangerous transplant, and with an exact sibling donor match has a 60 to 70 percent success rate.

Aplastic Anemia Causes

Aplastic anemia is a disorder where the body’s bone marrow is incapable of producing enough new cells to refill blood cells. The word aplastic suggests that the morrow in question, suffers from, what is called an aplasia. The aplasia, in this case, leaves the bone marrow unable to work properly. Anemia is the disorder where the body is not producing enough red blood cells, or hemoglobin. In typical cases of anemia, you will find that the patient has a low count of only red blood cells, but with aplastic anemia, the body is low in all three of the blood cells needed to function correctly; these are the white blood cells, the red blood cells, and the blood platelets.

All aplastic anemia causes are not known, but the main cause of this disorder is a type of autoimmune disorder. This disorder causes the white blood cells that start in the blood, to attack the body’s bone marrow. It is also said that aplastic anemia is brought on by the prolonged exposure to toxins like benzene. Many cases can also be linked to the use of some types of drugs, including carbamazepine, chlorampenicol, felbamate, phenytoin, phenylbutazone, and quinine. There have also been reports that aplastic anemia can be directly linked to severe radiation exposure from radioactive materials. There is also a 2 percent chance that patients diagnosed with viral hepatitis, will develop aplastic anemia.

The best way to effectively treat aplastic anemia is by undergoing a shutdown of the immune system. This is done by doctors medicating the patient on a daily basis, or by the patient undergoing, in severe cases, a full bone marrow transplant. The transplant can only be done if a matching donor can be found, and it should be known that there is a 50 percent survival rate in transplant cases such as this. This is because of the risk of infection, and the body’s inability to fight off such infections. If you are looking to know a bit more about aplastic anemia causes, it is suggested that you speak to your family doctor. Aplastic anemia is a very dangerous disorder, but there is a treatment for it.

Aplastic Anemia Treatment

Aplastic Anemia being a rare disease has led to many deaths mostly due to lack of early diagnosis. However, if the symptoms of the disease are discovered early enough, it is possible to either control or cure the disease completely. Just like any other disease, the severity of the condition determines the type of treatment to be undertaken. Cases of Aplastic Anemia could be severe, mild or moderate. Severe Aplastic Anemia requires that the patient be hospitalized for specialized treatment because it is life-threatening. In the initial stages of treatment, the patient may have to undergo blood transfusion as he or she awaits treatment. Different methods of treating Aplastic Anemia are available although the most commonly known standard methods are blood transfusion, bone marrow transplant and Immunosuppressive therapy. These treatments can either be used with an aim of managing the disease for a considerable time or to completely cure the disease.

Blood transfusion for example does not cure the disease and multiple blood transfusions may be required in order to provide the much needed blood cells that the bone marrow is unable to supply. The use of a growth factor may however be considered to help the body produce more blood cells to reduce the number of transfusions. Bone Marrow transplant is known to cure Aplastic Anemia and recurrence is usually limited to one out of five cases. A bone marrow transplant will often require that the patient finds a bone marrow donor, usually a close relative or an unrelated but matching donor. A likely risk in this kind of treatment is where the patient’s system rejects the transplant which may lead to serious complications. Immunosuppresants are used where the body immune system is the one attacking cells produced in the bone marrow. This treatment acts by suppressing the immune system. Examples of drugs used as immunosuppressants are anti-thymocyte globulin and clysporine. The disadvantage of these drugs is that they may weaken the patient’s immune system and in case the patient stops usage their conditions are likely to recur. Other methods used for containing the problem include the use of bone marrow stimulants and the use of anti-retrovirals and antibiotics.

Research on Aplastic Anemia is still ongoing to come up with more effective treatments for the disease. High-Dose Cyclophosphamide is among the most recent treatment for Aplastic Anemia in the market which is still being used in clinical trial. This medication is well known for its low relapse rate and fewer side effects as opposed to other types of chemotherapy and Aplastic Anemia treatments. Cytoxan is also used to refer to Cyclophosphamide which is actually a generic name. Patients who receive this treatment have to combine it with GCSF which is a growth factor. The Cytoxan eliminates the lymphocytes while leaving the healthy stem cells untouched. The bone marrow can then start producing new healthy cells for the body at the normal rate.