If you have aplastic anemia, your bone marrow’s production of blood cells decreases. This causes a reduction in the total number of cells in your bloodstream. This may occur gradually or suddenly. In most cases the cause of the problem cannot be identified. Sometimes the cause can be tentatively traced to exposure to a toxic substance such as benzene, certain substances used to dye hair, a drug taken for another disorder, or radiation. Most anticancer drugs produce similar changes in the bone marrow, but the condition usually improves when the drug is discontinued for awhile.

What are the Symptoms?

There are three main groups of symptoms. The decrease in production of red blood cells causes the symptoms of anemia . The decrease in production of granulocytes, a type of white blood cell, makes you more susceptible to infection. Finally, the decrease in platelet production leads to spontaneous bruising, red dots on the skin, and bleeding from the nose, mouth and other sites.

What are the Risks?

The main risks associated with aplastic anemia are infection and bleeding. Both of these may be severe enough to become life­threatening. You may improve spontaneousy or with treatment, but progressive failure of the bone marrow, worsening your condition, may also occur.

What Should be Done?

If you develop any of the symptoms described, see your physician at once. This is especially important if you are taking a drug or working with chemicals or radioactive materials. The physician will probably arrange for a blood test. If the test results show that aplastic anemia may be present, you may need to have a bone marrow biopsy, in which a small amount of bone marrow is removed and examined under the microscope. This examination should allow your physician to make a definite diagnosis.

What is the Treatment?

In cases where the disease is associated with a drug that is being taken for another problem, your physician will strongly consider stopping the drug and finding a suitable substitute. If there is any suspicion of continuing exposure to a toxic compound, you should remove yourself from contact with it.

Your physician will probably treat anemia and hemorrhage, or bleeding, with blood transfusions, and infections with antibiotics, which are usually given intravenously for best results. If you do not have an infection, but your granulocyte count is very low, you still may be given an antibiotic to reduce the chance of infection.

If the disease is severe or if it is growing worse, a bone marrow transplant offers the best hope of recovery. However, the bone marrow transplant has its own hazards. There is also a medication available, but it helps only a few patients.

The white blood cells known as neutrophils act as the body’s first defense against infections. Normally the neutrophils are produced in the bone marrow and are released into the bloodstream. In agranulocytosis, most or all of the neutrophils are destroyed, and there is a severe reduction in the number of neutrophils that are circulating in the blood. The result of this reduction in circulating neutrophils is decreased resistance to infection.The disease is often caused by a drug that you are taking for some other disorder. It can also be caused by a viral infection or by an antibody, or normally protective biochemical in your blood, that you develop against your own white blood cells. The disease may be the first sign of leukemia or aplastic anemia (previous article).

What are the Symptoms?

The characteristic symptom of the disease is susceptibility to infection. This is especially true in the mouth and throat, where ulcers often occur. Sometimes, if you have agranulocytosis, infections such as pneumonia progress unusually rapidly and are extremely severe, or even fatal.

What Should be Done?

If you have had one infection after another, see your physician, particularly if you are taking a prescription, VI even a non­prescription drug. Some drugs are known to carry a particular risk of damaging bone mar­row, and your physician will be alert to this possibility. The doctor will probably arrange for a blood test. If the results show that you may have agranulocytosis, a bone marrow biopsy, in which a small amount of bone marrow is removed and examined, will be necessary before your physician can make a definite diagnosis.

What is the Treatment?

Your physician will probably instruct you to stop taking any drug in case it is the cause of the disorder. If the level of granulocytes in your blood is very low, you will probably be given an antibiotic drug to prevent infection. If you already have an infection, or if you have a fever, you will probably be given antibiotics intravenously right away.

In most cases, the outlook for complete recovery from agranulocytosis is very good. Either the drug or the infection causing the disease is eliminated and recovery begins. Cases caused by one of the types of leukemia or aplastic anemia are more complex and so are those in which a difficult-to-control infection develops.

Information about the Condition

polycythemia Vera

What do doctors call this condition?

Primary polycythemia

What is this condition?

Polycythemia vera is a chronic bone marrow disorder characterized by increased red blood cell mass, increased white cell production, thrombocytosis, and increased hemoglobin concentration, with normal or increased plasma volume. It usually occurs between ages 40 and 60, most commonly among males of Jewish ancestry. It rarely affects children or Blacks and doesn’t appear to run in families.

The prognosis depends on the person’s age at diagnosis, the treatment used, and complications. The mortality rate is high if the disease is untreated or is associated with leukemia or other types of cancer.

What causes this condition?

In polycythemia vera, uncontrolled and rapid cell reproduction and maturation cause proliferation of all bone marrow cells. The cause of the uncontrolled cellular activity is unknown. Increased red blood cell mass causes blood thickening, which slows blood flow to small blood vessels. These conditions combine with thrombocytosis to promote blockages in some blood vessels.

What are its symptoms?

In its early stages, polycythemia vera usually produces no symptoms . However, as changes in circulation develop, the person may complain of a vague feeling of fUllness in the head, headache, dizziness, and other symptoms, depending on the body system affected. Paradoxically, hemorrhage may occur as a complication of polycythemia vera.

How is it diagnosed?

Lab studies confirm polycythemia vera by showing increased red blood cell mass and other characteristic findings. Bone marrow biopsy (removal and analysis of tissue) reveals increased levels of all bone marrow components.

How is it treated?

Phlebotomy (blood removal therapy) can reduce the red blood cell mass promptly. The frequency of phlebotomy and the amount of blood removed each time depend on the persons condition. Typically, 350 to 500 milliliters of blood can be removed every other day. After repeated phlebotomies, the person develops iron deficiency, which stabilizes red blood cell production and reduces the need for phlebotomy.

For severe symptoms, therapy to depress the bone marrow may be used. In the past, radioactive phosphorus or cancer drugs could usually control the disease. But these agents may cause leukemia and should be reserved for older people and those with problems uncontrolled by phlebotomy. Currently, the preferred myelosuppressive agent is the drug Hydrea. People who’ve had previous problems with blood clot formation should be considered for myelosuppressive therapy

Iron Deficiency Anemia

What is this condition?

In iron deficiency anemia, a lack of iron in the body causes an array of symptoms, including fatigue, weakness, and abnormally pale skin. When the body’s iron stores are low, the red blood cell count falls, and with it the supply of hemoglobin, the iron-containing pigment in red cells that carries oxygen. The blood’s oxygen-carrying capacity is thereby diminished.

A common disease worldwide, iron deficiency anemia affects 10% to 30% of adults in the United States. It’s most prevalent in premenopausal women, infants (especially premature or low-birth-weight infants), children, and adolescents (especially girls).

What causes it?

Iron deficiency anemia may result from:

• too little iron in the diet, as in prolonged unsupplemented breast­or bottle-feeding of infants or during periods of stress, such as rapid growth in children and adolescents

• iron malabsorption, as in chronic diarrhea, surgical removal of the stomach, and malabsorption syndromes

• blood loss caused by heavy menstrual bleeding, injury, gastrointestinal ulcers, cancer, drug-induced gastrointestinal bleeding, or twisted, dilated veins

• pregnancy, which diverts the mother’s iron supply to the fetus for red blood cell formation

• abnormal presence in urine of free (extracellular) hemoglobin.

What are its symptoms?

Because iron deficiency progresses slowly, many people have no symptoms at first (except those of any underlying condition). Typically, they don’t go to the doctor until anemia is severe. In advanced stages, they may have trouble breathing on exertion, fatigue, weakness, listlessness, pallor, inability to concentrate, irritability, headache, and susceptibility to infection. Also, the heart may pump more blood than usual and the pulse rate may increase.

Chronic iron deficiency anemia causes spoon-shaped and brittle nails, cracks in the corners of the mouth, a smooth tongue, and difficulty swallowing.

How is it diagnosed?

Blood tests and bone marrow studies may confirm iron deficiency anemia. However, test results may be misleading because of complicating factors, such as infection, pneumonia, blood transfusion, or iron supplements. Bone marrow studies may also provide important information about the disorder. The doctor also must rule out other forms of anemia.

How is it treated?

The first goal of treatment is to determine the underlying cause of anemia. Then, iron replacement can begin. The preferred treatment is an oral iron preparation or a combination of iron and ascorbic acid (which enhances iron absorption). However, some people may need iron injections - for instance, those who need more iron than they can take orally.

What is this Condition?

This disorder is a complication of conditions that accelerate blood clotting. It causes blockages in the small blood vessels, depletes the body’s supply of clotting factors and platelets, and destroys fibrin critical component of blood clots). In effect, the disorder causes severe bleeding at multiple sites.

Disseminated intravascular coagulation is generally an acute condition, but it may be chronic in people with cancer. The prognosis depends on early detection and treatment, on the severity of hemorrhaging, and on whether the underlying disease or condition can be treated.

What causes Disseminated Intravascular Coagulation?

Disseminated intravascular coagulation may result from a variety of disorders, but the reason for this isn’t known.

What are its symptoms?

The most significant symptom is abnormal bleeding in a person who has never had a serious bleeding disorder. The principal signs of such bleeding include oozing from the skin, red or purple skin spots, and hematomas caused by bleeding into the skin. Bleeding from sites of surgical or invasive procedures and from the digestive tract are equally significant, as are bluish, cold hands and feet and kidney problems.

Other possible symptoms include nausea, vomiting, shortness breath, decreased urination, seizures, coma, shock, failure of major body systems, and severe muscle, back, and abdominal pain.

How is it diagnosed?

Abnormal bleeding in the absence of a known blood disorder suggests disseminated intravascular coagulation. Lab findings that measure blood clotting support the diagnosis. Assessing kidney function may also provide important information.

Final confirmation of the diagnosis may be difficult because many of these test results also occur in other disorders. Additional diagnostic tests determine the underlying disorder.

How is it treated?

Successful management of disseminated intravascular coagulation depends on a prompt diagnosis and adequate treatment of the underlying disorder. Treatment may be supportive (when the underlying disorder is self-limiting, for example) or highly specific. If the person isn’t actively bleeding, supportive care alone may reverse the disorder. However, in active bleeding the doctor may order intravenous heparin and transfusions of blood, fresh-frozen plasma, platelets, or packed red blood cells.

Chronic granulocytic leukemia begins in the same way as acute myelogenous leukemia . A malignant, or life-threatening, change occurs in bone marrow cells that pro­duce granulocytes, a type of white blood cell. As a result, the number of granulocytes in your blood rises excessively, often to between 20 and 40 times the normal level. When the disease is not treated, the multiplication of granulocytes may limit the production of red blood cells, so you may also become anemic. In addition, the accumulation of leukemic cells may cause enlargement of both your spleen and your liver.

What are the symptoms?

If you have chronic granulocytic leukemia, you feel generally ill, have little appetite, and lose weight. You may have a fever and sweat at night. In addition, your enlarged spleen may cause a sense of fullness in the left upper portion of the abdomen. You may also have symptoms of anemia .

What are the risks?

If chronic granulocytic leukemia is not treated, it is likely to be fatal within weeks or months. The disease usually responds very well to initial treatment, which gives most patients at least two to three more years of fairly normal life. Eventually, the disease begins to resemble acute leukemia, and it no longer responds to treatment.

What should be done?

If you have any of the symptoms described, you should see your physician, who will probably examine you and arrange for blood tests. The blood tests will either rule out the disease, or indicate the need to take further blood tests. To establish a clear diagnosis, there may also be a bone marrow biopsy, in which a small sample of marrow is removed to be examined.

What is the treatment?

Most people who have the disease can be treated as out-patients. The basic treatment is tablets of anticancer drugs that usually restore bone marrow production to normal and clear up the symptoms. Some people need to continue to take the medication regularly, while others require it only intermittently. Your physician will watch your condition, and take blood tests every two to four weeks. This is important because the dose of medication often needs to be adjusted, and too much of the drug decreases your blood count to dangerous levels.

After some time, treatment with drugs will no longer control the disease. The average length of time before this occurs is three years, but it can be much shorter or much longer. Eventually the leukemia becomes much worse and you will probably be admitted to a hospital. At this stage of the disease, stronger anticancer drugs may help you for a short time, but often it is only a matter of weeks before the disease proves fatal.