Pure Red Cell Aplasia

Pure red cell aplasia (PRCA) is a rare blood disorder characterized by the near or complete absence of red blood cells (RBCs) in the body. RBCs are the cells in the blood that carry oxygen to the other parts of the body. As a result of this disorder, anemia develops from the lack of oxygen-carrying RBCs. The exact cause of this disorder is unknown, though it has been linked to certain autoimmune diseases, medications, and viruses.

PRCA can occur at any age and in both sexes. Symptoms of the disorder usually develop slowly, and may include fatigue, paleness, shortness of breath, rapid heart rate, and dizziness. Diagnosis of PRCA is done through blood tests and imaging examinations such as X-rays. Treatment includes corticosteroids, antithymocyte globulin, and human intravenous immunoglobulin.

Causes of PRCA

The exact cause of PRCA is unknown. It is believed to be associated with certain autoimmune diseases, medications, and viruses. Development of PRCA has been linked to such autoimmune diseases as systemic lupus erythematosus and Sjögren’s syndrome. Use of certain medications, such as those used to treat hypertension, is also believed to be a factor in PRCA.

Symptoms

The main symptom of PRCA is anemia, which results from the lack of oxygen-carrying red blood cells. Other symptoms may include fatigue, paleness, shortness of breath, rapid heart rate, and dizziness. These symptoms may develop slowly over time.

Diagnosis

PRCA is diagnosed with blood tests, which are used to measure the number of red blood cells, white blood cells, platelets, and hemoglobin in the body. Imaging examinations such as X-rays can also be used to confirm the diagnosis. In some cases, a bone marrow biopsy may be done to evaluate the condition of the bone marrow.

Treatment

Treatment for PRCA generally depends on the severity of the symptoms and the underlying cause. Treatment options may include:

Corticosteroids: These drugs are used to combat the body's autoimmune response.
Antithymocyte globulin: These medications help to suppress the immune system and reduce inflammation.
Human intravenous immunoglobulin: This is a form of immunotherapy that helps to boost the immune system.
Plasmapheresis: This is a procedure in which the blood is filtered to remove antibodies.
Blood transfusions: These are used to replace lost red cells and help treat anemia.

In some cases, a bone marrow transplant may be recommended, but this treatment is only performed in rare cases.