Pure Red Cell Aplasia (PRCA)

What is pure red cell aplasia (PRCA)?

Pure red cell aplasia (PRCA) is a rare blood disorder that happens when your bone marrow doesn’t produce the normal number of red blood cells. When your red blood cell supply drops below normal levels, you have anemia. With anemia, you don’t have enough healthy red blood cells carrying oxygen to your tissues and organs.

What are pure red cell aplasia symptoms?

PRCA causes common anemia symptoms, which may include:

• Fatigue.
• Difficulty breathing (dyspnea).
• Dizziness.
• Skin that’s paler than usual.
• Headaches.
• Pulsatile tinnitus.
• Angina.

What causes PRCA?

Pure red cell aplasia has several different causes. People who develop PRCA often have underlying medical conditions. It may also be a side effect of medications.

People can inherit PRCA or develop it because they have certain medical conditions. For example, people with Diamond-Blackfan anemia, an inherited anemia that’s usually diagnosed in childhood, may develop pure red cell aplasia. Other possible causes include:

• Autoimmune diseases: Conditions like systemic lupus erythematosus, rheumatoid arthritis and inflammatory bowel disease are examples of autoimmune diseases that may cause PRCA.
• Blood cancer: Chronic lymphocytic leukemia (CLL) and large granular lymphocytic leukemia (LGL) are two of the most common blood cancers linked to PRCA.
• Cancerous tumors: Thymoma (thymic carcinoma) is the most common cancerous tumor linked to pure red cell aplasia.
• Viral infections: Parvovirus B19 infection is a common cause of PRCA. It affects people who have weak immune systems or other underlying condition that affects their bone marrow. This virus attacks stem cells in your bone marrow that are intended to become red blood cells. Other infections include human immunodeficiency virus (HIV), Epstein-Barr virus and all types of hepatitis.
• Bacterial infections: Group C streptococcus, tuberculosis and bacterial sepsis may cause PRCA.
• Medication: Many different medications are linked to PRCA. For example, erythrocyte-stimulating agents (ESA) may cause PRCA. ESAs help your body produce more red blood cells.
• Pregnancy: Some people develop PRCA during pregnancy. The condition goes away after giving birth.

What are the risk factors for this condition?

Risk factors include carrying the inherited genetic mutation that causes Diamond-Blackfan anemia, having certain kinds of cancer and developing specific viral and bacterial infections.

How do healthcare providers diagnose pure red cell aplasia?

Providers diagnose this condition by doing a physical examination, blood tests and imaging tests:

• Complete blood count (CBC), including a count of white blood cell types.
• Reticulocyte count.
• Peripheral blood smear.
• Parvovirus B19 test.
• Computed tomography (CT) scan.
• Bone marrow aspiration or bone marrow biopsy.

How do healthcare providers treat pure red cell aplasia?

Treatment varies based on what caused you to develop PRCA. Options include:

• Immunosuppressants: These medications help control your immune system. Common medications for PRCA include cyclosporine (Gengraf®, Neoral® or Sandimmune®) and corticosteroids (prednisone). You may need to stay in the hospital during treatment and you may need more than one treatment.
• Blood transfusion: Receiving donor blood followed by treatment to remove excess iron that could damage your organs.
• Surgery:If a thymus tumor causes PRCA, surgeons may remove your thymus (thymectomy) to prevent the tumor from spreading.
• Stem cell transplant: People with Diamond-Blackfan anemia may need stem cell transplants to replace unhealthy bone marrow and blood cells.
• Immunoglobulin therapy: If you have PRCA because you have parvovirus B19 infections, providers may use immunoglobulin therapy to treat the virus so it stops damaging your immature red blood cells.

What are treatment side effects?

Side effects vary depending on the treatment. Your healthcare provider will inform you of these side effects before you begin treatment. They’ll also help you manage treatment side effects.

Immunoglobulin therapy side effects

Providers use immunoglobulin therapy to treat parvovirus B19. Side effects include:

• Headache.
• Dizziness.
• Muscle cramps.
• Joint pain.
• Pain, swelling and redness where the drug was injected.
• Kidney issues.

Immunosuppressant side effects

Side effects vary depending on the type of immunosuppressant. These drugs can cause:

• Acne.
• Diabetes.
• Fatigue.
• Hair loss or growth.
• Headaches.
• Trouble concentrating or remembering.
• High blood pressure.
• Mouth sores.
• Thinning bones (osteoporosis).
• Tremors.
• Weight gain.
• Upset stomach and nausea and vomiting.

Blood transfusion side effects

Providers may use blood transfusions if other treatments haven’t been effective. Fever and allergic reactions such as rashes or hives are common blood transfusion side effects. Less common side effects may include:

• Delayed transfusion reaction.
• Infections.
• Red blood cell antibodies.
• Iron overload. Some people retain iron in their organs. Excess iron may damage organs. Providers treat iron overload with chelation therapy to remove the excess iron.

Surgery side effects

All surgeries come with certain risks and side effects. Common surgery side effects may include:

• Reaction to anesthesia.
• Excessive bleeding.
• Infection.

How soon after treatment will I feel better?

This depends on the treatment. People who take corticoid steroids often begin feeling better within a few weeks. People who have a thymectomy may need more time to recover.

Can I prevent PRCA?

No. Pure red cell aplasia happens for many different reasons, including underlying conditions that you can’t prevent.

What is the life expectancy for someone with PRCA?

This depends on what caused you to develop PRCA, the treatment and how well you responded to treatment. Life expectancy for someone who developed PRCA because they have chronic lymphocytic leukemia (CCL) may be different from someone who has PRCA because they have parvovirus B19.

Some studies show people with acquired PRCA live between 12 and at least 14 years after diagnosis. In general, people with this condition don’t live as long as the general population. It’s important to remember that survival rates and other data are estimates, not predictions. If you have PRCA, ask your healthcare provider about your prognosis or expected outcome.

What’s it like to live with PRCA?

In a sense, living with pure red cell aplasia is like living with a chronic illness. That’s because PRCA can come back. To prevent that, you may need regular treatment for the rest of your life. Treatment may include:

• Immunosuppressants: Many people will need to take maintenance doses to avoid relapse, or a second case of PRCA.
• Blood transfusions: Some people may need repeated blood transfusions and chelation therapy.
• Continuous follow-up: People with PRCA have an increased risk of infection and organ failure. If you have this condition, you should expect to see healthcare providers regularly so they can monitor your overall health and check for PRCA recurrence or complications.

How do I take care of myself?

Following up with your healthcare providers is one of the best ways to take care of yourself. While treatment may have eliminated anemia symptoms, PRCA may come back. You may need additional treatment to reduce the risk of PRCA returning. People at risk for anemia sometimes benefit by:

• Following a healthy diet. Poor diet is the primary reason why people develop anemia. Ask your healthcare provider about iron-rich foods and other foods you should eat.
• Drinking enough water to stay hydrated.
• Getting regular exercise. Check with your healthcare provider about ways to exercise safely.
• Washing your hands often to avoid infection. You may also want to ask your healthcare provider about vaccines that defend against common infections.
• Tracking your symptoms and letting your healthcare provider know about any changes.

When should I see my healthcare provider?

Contact your healthcare provider any time you notice changes in your body that may be symptoms of anemia. If you’re receiving treatment for an underlying condition, contact that specialist if you notice your symptoms are getting worse.

What questions should I ask my healthcare provider?

Here are some questions you may want to ask your healthcare provider about pure red cell aplasia:

• Why did I develop this disease?
• What are treatments?
• What are treatment side effects?
• Will I need to stay in the hospital for treatment?
• Will treatments cure the condition?
• Will I need to take medication for the rest of my life?

A note from Cleveland Clinic

Pure red cell aplasia (PRCA) is a rare condition that happens for many different reasons. You may have PRCA because you have an autoimmune disorder or cancer. Some people develop PRCA because they were exposed to a common virus. Healthcare providers can treat pure red cell aplasia and eliminate anemia symptoms. That’s great news, of course, but PRCA can come back. You may need lifelong treatment to reduce that risk. If you have pure red cell aplasia, ask your healthcare provider what you can expect. They’ll explain any ongoing treatment you may need.