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Idiopathic aplastic anemiaDefinitionIdiopathic aplastic anemia is failure of the bone marrow to properly make all types of blood cells for an unknown reason. The bone marrow is the soft tissue inside bones that helps form blood cells. See also: Alternative NamesAnemia - idiopathic aplasticCausesAplastic anemia results from injury to the blood stem cell, a cell that develops into other blood cell types. The injury causes a reduction in the number of every type of blood cell in the body -- red cells, white cells, and platelets. This reduction is called pancytopenia. Idiopathic means the cause is unknown. However, idiopathic aplastic anemia is thought to occur when the body reacts against it's own cells. This is called an autoimmune disorder. The disease may be acute or chronic, and may be slowly get worse. There are no known risk factors. Aplastic anemia may also be caused by certain medical conditions (such as pregnancy or lupus) or exposure to some toxins or drugs (including chemotherapy). See: Secondary (acquired) aplastic anemia In some cases, aplastic anemia is associated with another blood disorder called paroxysmal nocturnal hemaglobinuria (PNH). SymptomsSymptoms are the result of bone marrow failure and the loss of blood cell production. Low red cell count (anemia) leads to fatigue and weakness. Low white cell count (leukopenia) causes an increased risk of infection. Low platelet count (thrombocytopenia) results in bleeding, especially of mucus membranes and skin. General symptoms include:
Exams and Tests
TreatmentMild cases of aplastic anemia may be treated with supportive care or may require no treatment. In moderate cases, blood transfusions and platelet transfusions will help correct the abnormal blood counts and relieve some symptoms. Severe aplastic anemia, which shows up as a very low blood-cell count, is a life-threatening condition. Bone marrow transplant is recommended for severe disease in younger patients. For older patients, or for those who do not have a matched bone marrow donor, horse antithymocyte globulin (Atgam) or rabbit antithymocyte globulin (thymoglobulin) are alternative treatments. These medicines suppress the body's immune system, allowing the bone marrow to once again make blood cells. Atgam may be used with another drug, such as cyclosporine. Other medications to suppress the immune system, such as cyclophosphamide (Cytoxan) or corticosteroids, may be also used. Outlook (Prognosis)Untreated aplastic anemia is an illness that leads to rapid death. Bone marrow transplant has been successful in young people, with long term survival of 80%. Older people have a survival rate of 40 - 70%. Possible Complications
When to Contact a Medical ProfessionalCall 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. PreventionThere is no known prevention for idiopathic aplastic anemia. ReferencesGoldman L, Ausiello D, eds. Cecil Textbook of Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007: pp.1241-6.
Review Date:
7/11/2008 Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional 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. © 1997-
A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.  |
