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Idiopathic aplastic anemia Causes and Risks:
Idiopathic aplastic anemia is a condition that results from injury to the stem cell, a cell that gives rise to other cell types when it divides. Consequently, there is a reduction in all cell types--red blood cells, white blood cells and platelets--with this type of anemia , which is called
pancytopenia. The cause of idiopathic aplastic anemia is unknown, but is thought to be an autoimmune process (when the body reacts against its own cells). Causes of other types of aplastic anemia may be chemotherapy , radiation therapy , toxins , drugs, pregnancy , congenital disorder, or systemic lupus erythematosus .
Symptoms arise as the consequence of bone marrow failure. Anemia (low red blood cell count ) leads to fatigue and weakness . Low white blood cell counts, or neutropenia, causes an increased risk of infection. Low platelet counts, or thrombocytopenia , results in bleeding of mucus membranes and skin. The disease may be acute or chronic , and is always progressive. Risk factors are unknown. The incidence is 2 out of 1 million people.
Prevention:
There is no known prevention for idioplastic anemia .
Symptoms:
Signs and Tests:
A physical examination reveals an enlarged spleen , tenderness of the sternum, and irregular heart rate .
Tests:
Treatment:
Mild cases of aplastic anemia are treated with supportive care. Blood transfusions and platelet transfusions help correct the abnormal blood counts and relieve some symptoms.
Severe aplastic anemia, as evidenced by very low blood cell counts, is a life-threatening condition. Bone marrow transplant for people 30 and under is indicated for severe disease. For adults over 40, or for those who do not have a matched bone marrow donor, antithymocyte globulin (ATG) is the alternative treatment. ATG is a horse serum that contains antibodies against human T cells and is used in an attempt to suppress the body's immune system, allowing the bone marrow to resume its blood cell generating function. Other medications to suppress the immune system may be used, such as cyclosporine.
Prognosis:
Untreated aplastic anemia is an illness that leads to rapid death. Bone marrow transplantation has been successful in young people, with long term survival of 80%. Older people have a survival rate of 40 to 70%.
Complications:
Complications of treatment may lead to rejection of bone marrow graft, or severe reaction to ATG.
Call Your Healthcare Provider:
Call your health care provider or go to the emergency room if bleeding occurs for no reason or if bleeding is difficulty to stop.
A small amount of bone marrow is removed during a bone marrow aspiration. The procedure is uncomfortable, but can be tolerated by both children and adults. The marrow can be studied to determine the cause of anemia, the presence of leukemia or other malignancy, or the presence of some "storage diseases" in which abnormal metabolic products are stored in certain bone marrow cells.
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