What are the other Names for this Condition? (Also known as/Symptoms)

• Acquired Pure Red Cell Aplasia
• PRCA (Pure Red Cell Aplasia)
• Pure Red Blood Cell Aplasia

What is Pure Red Cell Aplasia? (Definition/Background Information)

• A typical life span of a red blood cell ranges from approximately 100 to 120 days. The red blood cells get released from the bone marrow and circulate in the body to deliver oxygen
• Pure Red Blood Cell Aplasia (PRCA) is an uncommon condition that results due to a defect in the bone marrow
• PRCA arises when damage occurs to the bone marrow, where it is unable to produce a sufficient amount of red blood cells. As a result, there is a severely reduced number of circulating red blood cells, consequently affecting oxygen availability in the body
• Pure Red Blood Cell Aplasia could occur as a result of a defect at birth, or develop over time associated with an autoimmune disorder, or as a transient and reversible condition following viral infections or medications
• The signs and symptoms of PRCA include weakness, anemia, susceptibility to infections, anemia, etc. A physician may diagnose the condition by a thorough physical examination, assessment of family and/or medical history, monitoring heart and lung function, bone marrow function, etc.
• Treatment options could include the administration of steroids, immunosuppressant, etc. However, the prognosis of PRCA depends upon the severity of the condition

Who gets Pure Red Cell Aplasia? (Age and Sex Distribution)

• Pure Red Blood Cell Aplasia may be seen as a congenital or acquired condition that may affect any individual of any age group
• It is seen among both men and women, although autoimmune-related abnormalities are more likely to be seen in women
• There are no current studies that demonstrate the condition being more predominant in a specific set of race, nor are there any reports to indicate that it is more common among a particular ethnic group

What are the Risk Factors for Pure Red Cell Aplasia? (Predisposing Factors)

The risk factors reported for Pure Red Blood Cell Aplasia may include:

• A strong family history for the condition.
• Use of certain medication by the mother
• Pre-existing illness of the mother, leading to an undernourished fetus
• Viral fever
• Certain medications
• Autoimmune disorders

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pure Red Cell Aplasia? (Etiology)

Generally, Pure Red Blood Cell Aplasia occurs as a result of an immunological defect/injury to the bone marrow, as a consequence of defective early-phase maturation of erythrocytes (red blood cells).

• It may arise due to a defect at birth too
• PRCA could be acquired over time as a result of an underlying autoimmune condition, such as systemic lupus, and some types of cancer (Example - thymoma)
• Use of certain medications, such as anti-erythropoietin antibodies, anti-seizure medicines, chloramphenicol, etc., could lead to PRCA
• Drug use/abuse is also a reported cause of PRCA
• Viral infections (such as mumps, viral hepatitis, etc.) could lead to a transient form of PRCA as well

In some cases, the cause of PRCA is unknown (idiopathic), with the individual having no prior history or symptoms for it.

What are the Sign and Symptoms of Pure Red Cell Aplasia?

The signs and symptoms associated with Pure Red Cell Aplasia may include:

• Generalized weakness in the body
• Changes in skin tone
• Excessive bleeding that may lead to easy bruising
• Heart conditions that may lead to the development of a stroke
• Anemia
• Developing an infection 
• Cough
• Changes in eye color (yellow)
• Negative effect on the growth spurt

How is Pure Red Cell Aplasia Diagnosed?

A physician may employ one or more of the following methods to arrive at an accurate diagnosis of Pure Red Blood Cell Aplasia:

• Patient history and physical examination to assess and understand the cause
• Evaluating the function of the extremities and testing how alert the individual is
• Examination of the heart and lungs to identify the presence of any abnormalities
• Tests to check for abnormalities with the immune response
• Tests to evaluate bone marrow function
• Cultures to identify the presence of any infection

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Pure Red Cell Aplasia?

The possible complications associated with Pure Red Cell Aplasia may include:

• Developing other illnesses
• Excessive bleeding which may lead to easy bruising
• Infections that may cause organ dysfunction or failure

How is Pure Red Cell Aplasia Treated?

The treatment of Pure Red Cell Aplasia may be undertaken as:

• If the cause of Pure Red Cell Aplasia is an autoimmune-related disorder, medications such as steroids can be provided to help reduce the symptoms
• Other medications, such as drugs that will suppress the immune response, could be administered to help the individual overcome the condition
• Discontinuing medications that are known to cause PRCA
• Treatment of underlying conditions that have led to PRCA
• Treatment of infections
• Blood transfusions in cases of severe anemia

How can Pure Red Cell Aplasia be Prevented?

When Pure Red Cell Aplasia is an inherited disorder, it develops during very early stages of life, including the fetal stage. When this happens, the baby usually presents with symptoms at birth. Being able to identify it early in life will help with treatment, to keep the condition either suppressed or maintained so that it does not develop into a chronic form or give rise to other health conditions.

• Early genetic testing could help prepare the parents become aware of the situation prior to the birth of the baby and be counseled on what needs to be done
• PRCA as an illness associated with other conditions could potentially be prevented by treating the underlying cause(s)
• Avoiding medications and drugs that could potentially lead to PRCA could help prevent the disease from developing

In many cases, Pure Red Cell Aplasia is not a preventable condition.

What is the Prognosis of Pure Red Cell Aplasia? (Outcomes/Resolutions)

• The prognosis of Pure Red Cell Aplasia varies depending on its severity.
• When present at birth, PRCA could be treated with medications to reduce the symptoms and help with the poor immune system; however, there is an increased probability for the condition to recur
• Transient PRCA resolves itself after the withdrawal of medication or being cured of the viral infection, with a good prognosis

Additional and Relevant Useful Information for Pure Red Cell Aplasia:

Pure Red Blood Cell Aplasia, Acquired, usually presents as a severe condition. However, if it is properly managed from the early stages, it can be maintained and prevented from developing into its chronic form.