Bone marrow aspiration
Bone marrow aspiration
Antibodies
Antibodies

Idiopathic aplastic anemia

Definition:
Idiopathic aplastic anemia is a failure of the bone marrow to properly form all types of blood cells.

Alternative Names:
Anemia - idiopathic aplastic

Causes, incidence, and risk factors:

Idiopathic aplastic anemia is a condition that results from injury to the stem cell, a cell that gives rise to other cell types after 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 (the body reacting 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 count, or leukopenia, causes an increased risk of infection. Low platelet count, or thrombocytopenia, results in bleeding, especially of mucus membranes and skin.

The disease may be acute or chronic, and is almost always progressive. Risk factors are unknown.

Symptoms:
Signs and 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 is indicated for severe disease in younger patients.

For older patients, 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.

Expectations (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:

Severe infections or bleeding may develop.

Complications of treatment may include graft failure or graft vs. host disease, and an autoimmune disease, after bone marrow transplant. Patients can have reactions to ATG.

Calling your health care provider:
Call your health care provider or go to the emergency room if bleeding occurs for no reason or if bleeding is difficult to stop. Call if you notice frequent infections or unusual fatigue.
Prevention:
There is no known prevention for idiopathic aplastic anemia.

Review Date: 10/6/2001
Reviewed By: Rebecca Elstrom, M.D., University of Pennsylvania Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org). URAC's accreditation program is the first of its kind, requiring compliance with 53 standards of quality and accountability, verified by independent audit. A.D.A.M. is among the first to achieve this important distinction for online health information and services. A.D.A.M. is also a founding member of Hi-Ethics (www.hiethics.com) and subscribes to the principles of the Health on the Net Foundation (www.hon.ch).

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2003 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.