Pure Red Cell Aplasia (PRCA)

Pure red cell aplasia (PRCA) is when your bone marrow stops making red blood cells, and you develop severe anemia. PRCA symptoms include fatigue and a rapid heart rate. Your skin may be paler than usual. Autoimmune diseases, infections and some types of cancer increase the chance you’ll develop pure red cell aplasia.

Types of pure red cell aplasia

There are three types:

• Congenital PRCA: The inherited disorder Diamond-Blackfan anemia causes this type. Congenital means the condition is present at birth.
• Primary acquired PRCA: This type happens when your immune system attacks red blood cells forming in your bone marrow. Healthcare providers don’t know why this happens.
• Secondary PRCA: You have a known medical condition that leads to pure red cell aplasia. Healthcare providers may refer to this as acquired PRCA. This is the most common type. It’s very rare, affecting 1 in 1 million people.

Symptoms of PRCA

Symptoms may develop slowly. It may be several weeks or months before you notice changes in your body. Your symptoms may be mild and slowly get worse. Common symptoms may include:

• Chest pain
• Fatigue
• Difficulty breathing (dyspnea)
• Dizziness
• Skin that’s paler than usual
• Headaches
• Pulsatile tinnitus, which is a whooshing sound in your ears

Condition causes

Many things may cause PRCA. You may inherit a disorder that causes it (congenital PRCA). It can happen if your immune system attacks your body instead of defending it. You can have health issues that lead to it. This is acquired or secondary pure red cell aplasia. Certain medications may also cause it. But often, pure red cell aplasia develops for no known reason. Examples of acquired or secondary PRCA are:

Autoimmune diseases

In an autoimmune disease, your immune system mistakenly attacks your blood, organs or tissues. Examples of autoimmune disorders that lead to PRCA are:

• Lupus
• Rheumatoid arthritis
• Inflammatory bowel disease
• Hypogammaglobulinemia

Lymphoproliferative disorders

Lymphoproliferative disorders (LPDs) are diseases that affect lymphocytes, a type of white blood cell. These cells help your immune system fight intruders. Some LPDs affect how your immune system reacts. Others happen when abnormal lymphocytes multiply uncontrollably and outnumber your red blood cells. LPDs that cause pure red cell aplasia include:

• Chronic lymphocytic leukemia
• Chronic myelogenous leukemia
• Large granular lymphocytic leukemia
• Hodgkin/non-Hodgkin lymphoma
• Monoclonal gammopathy of unknown significance (MGUS)

Cancerous tumors

Thymoma and thymic carcinoma are cancerous tumors that start in your thymus.

Medication

Several medications have a link to PRCA, including:

• Lab-made form of erythropoietin, a hormone that treats anemia
• Certain antiseizure medications
• Certain antiviral medications
• Immune checkpoint inhibitors, immunomodulator medications that treat cancer

Viral infections

Viral infections are a common cause of PRCA, including:

• B19 parvovirus
• HIV
• Viral hepatitis (A, B, C or E)
• Epstein-Barr virus

Very rarely, bacterial infections may cause PRCA.

Other causes

Some women develop PRCA during pregnancy. The condition often goes away after you give birth. It can also happen if you have a stem cell transplant that uses stem cells from someone whose blood type is different from yours.

How doctors diagnose this condition

A healthcare provider will do a physical exam. They’ll ask about your symptoms. They may ask about your health history. Tests may include:

• Blood tests: Common blood tests are complete blood count (CBC), reticulocyte count and peripheral blood smear. These tests check for issues with your red blood cells. You may have a parvovirus B19 test.
• Imaging tests: Your provider may do a CT scan if they suspect a cancerous tumor is causing PRCA.
• Bone marrow aspiration: Your provider may also do a bone marrow biopsy. This test may show whether you have low levels of young red blood cells.

How is PRCA treated?

A healthcare provider will plan treatment to manage the condition that causes PRCA. Treatments may include:

• Blood transfusion: This treatment helps with anemia symptoms like fatigue and shortness of breath.
• Immunoglobulin therapy: This treatment targets parvovirus B19. It keeps the virus from causing more damage to red blood cells in your bone marrow.
• Immunosuppressants: These medications may control your immune system. Common medications for PRCA include cyclosporine (Gengraf^®, Neoral^® or Sandimmune^®) and corticosteroids (prednisone).
• 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.

When should I seek care?

PRCA symptoms start out mild and slowly get worse. Contact a healthcare provider if you have symptoms like fatigue, weakness or notice your skin is paler than usual.

What is the life expectancy for someone with this condition?

How long you may live with this condition depends on factors like:

• The condition that led to PRCA
• The severity
• Your age when you first had symptoms
• If treatment improved or eliminated your symptoms

Your situation may be different from others who have PRCA. Your healthcare provider is the best person to explain what you can expect.

Is there anything I can do to feel better?

Medication and other treatments may help manage pure red cell aplasia. You can help support your health in these ways:

• Protect yourself from infection: Wash your hands often, avoid people who are sick and ask your provider about shots that defend against common infections.
• Keep track of your symptoms: Let your provider know about any changes in your health. They may be signs that PRCA is coming back.