7716 - Aplastic anemia

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Acronym: AA

Definition

Aplastic anemia is a diverse group of bone marrow disorders characterized by a pancytopenia, due to reduction of development of blood cells, marrow cells, and their replacement by fat. Granulocytopenia and thrombocytopenia often accompany the condition.

Etiology

The exact cause of aplastic anemia is unknown. The anemia is caused by deficient red blood cell (RBC) production due to bone marrow disorders. The disorder may be congenital, or appear later in life, or it may be inherited. Causes of the condition may include the following:

  • drug use

  • toxic agents

  • radiation

  • immunologic factors

  • severe diseases such as:

    • hepatitis

    • preleukemia

    • neoplastic infiltration of the bone marrow

Signs & Symptoms

Signs and symptoms of the condition may include:

  • an insidious onset

  • severe anemia, including pancytopenia

  • a waxy pallor of the skin and mucous membranes

  • brown skin pigmentation

  • bleeding into the skin and mucous membranes

  • an infection without characteristic signs and symptoms of inflammation

  • progressive weakness

  • fatigue

  • shortness of breath

  • headache

  • pallor

  • tachycardia

  • congestive heart failure

There may be ecchymosis, petechiae, and hemorrhage, especially of the mucous membrane. The areas usually affected by the hemorrhage include the nose, gums, rectum, and vagina, retina, or central nervous system (CNS). 

Tests

The diagnosis of aplastic anemia requires aspiration of bone marrow and biopsy examinations. Other tests performed to diagnose the condition include:

  • a complete blood count (CBC) which may show agranulocytosis, red blood cells which are normochromic-normocytic, a reduced white blood cell count (WBC), and reduced platelets

  • routine throat, urine, and blood cultures to check for infections

  • serological tests for hepatitis, infectious mononucleosis, and HIV

Treatment

Treatment is directed toward eliminating any identifiable causes. Treatment measures may include:

  • the administration of packed cells and platelets

  • leukocyte transfusions

  • bone marrow transplantation for anemia resulting from severe aplasia and for those persons needing constant red blood cell (RBC) transfusion

  • antibiotics (for infection)

  • oxygen therapy

  • marrow-stimulating agents such as androgens, immunosuppressants, and colony-stimulating factor agents

Residuals

Frequent bleeding and repeated infections are likely to occur, which are characterized by acute, recurring episodes. There may be weakness and fatigue which would require regular rest periods. Oxygen therapy may be needed, and long term treatment can be expected.

Special Considerations

Notes

  • A 100 percent evaluation for peripheral blood or bone marrow stem cell transplant shall be assigned as of the date of hospital admission and shall continue with a mandatory VA examination six months following hospital discharge. Any change in evaluation based upon that or any subsequent examination shall be subject to the provisions of 38 CFR 3.105(e).

  • The term "newer platelet stimulating factors" includes medication, factors, or other agents approved by the United States Food and Drug Administration.