APLASTIC ANEMIA

ALTERNATE NAMES

Acquired Aplastic Anemia; Anemia Aplastic; Bone Marrow Failure; Idiopathic Aplastic Anemia; Secondary Aplastic Anemia; Severe Aplastic Anemia

DESCRIPTION

Aplastic Anemia (AA) is a rare blood disorder that occurs when the body’s bone marrow does not make enough new cells to replenish blood cells. Although anemia usually refers to low red blood cell counts, typically in AA there are also low white blood cell and low platelet counts. In most cases, AA is considered to be idiopathic, meaning the cause is unknown. Less commonly, AA can be caused by exposure to certain drugs, viral infections (i.e. hepatitis, Epstein-Barr virus, cytomegalovirus, parvovirus B19, and HIV), autoimmune disorders (i.e. lupus and rheumatoid arthritis) radiation therapy, or toxins (i.e. pesticides, benzene or arsenic). Symptoms of AA may include bleeding of the gums, bleeding of internal organs, bruising, frequent or severe infections, nosebleeds, rapid heart rate, rash, shortness of breath during physical activity, and weakness.

DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM CODING

Diagnostic testing: Bone marrow aspirate and biopsy is required for diagnosis and to rule out other causes of pancytopenia such as cancer or infection; blood counts documenting anemia, thrombocytopenia, leukopenia and low reticulocytes counts.

Physical findings: Anemia with fatigue and pallor, bruising, petechiae (non-raised hemorrhage in the skin or in a membrane), and bleeding gums.

ICD-9: 284.9

ONSET AND PROGRESSION

AA usually gets worse unless the cause is removed or the disease is treated. Mild and moderate forms of the disease often progress slower than the severe form.

TREATMENT

Supportive treatments for AA include blood transfusions, platelet transfusions, and medications such as antithymocyte globulin (ATG) or antilymphocyte globulin (ALG), cyclosporine, or low dose chemotherapy. If the bone marrow does not recover and start producing blood cell elements, then bone marrow transplant (BMT) is required. BMT has been successful in the treatment of young patients, with a long-term survival of approximately 80%. Older patients have a survival rate of 40 – 70% after a transplant. If left untreated, severe aplastic anemia can become life threatening.

SUGGESTED PROGRAMMATIC ASSESSMENT*

Suggested MER for evaluation:

• Clinical history and examination that describes the diagnostic features and physical findings.

• Laboratory studies and bone marrow biopsy pathology reports.

• Documentation of bone marrow or stem cell transplantation.

Suggested Listings for Evaluation:

DETERMINATION

LISTING

REMARKS

Meets Listing

7.17, 107.17

Listing level severity requires documentation of bone marrow or stem cell transplantation; reevaluate 1 year after transplantation.

Equals Listing

7.17, 107.17