Pure red cell aplasia (PRCA)

Find out about pure red cell aplasia (PRCA): symptoms, diagnosis, treatment and sources of further information and support.

What is pure red cell aplasia (PRCA)?

Pure red cell aplasia (PRCA) is a rare type of anaemia, a condition characterised by a shortage of red blood cells.

It affects around 1 in 500,000 people who are treated with the hormone erythropoietin (EPO) for anaemia caused by chronic kidney disease. Men and women are affected equally.

What are the signs and symptoms of PRCA?

Common symptoms include:

  • increased tiredness
  • pale skin
  • breathlessness
  • feeling faint.

These are all signs of anaemia and can slowly become more noticeable over time.

What causes PRCA?

There are three main causes of PRCA.

  • Congenital – where the condition is inherited from the parents. This is also known as Diamond-Blackfan anaemia.
  • Acquired – where it occurs by itself (primary autoimmune PRCA)
  • Secondary – where it occurs as a result of another condition, including autoimmune diseases like lupus or rheumatoid arthritis, some types of cancer, a viral infection like hepatitis or as a result of a reaction to medication.

The kidneys produce a hormone called erythropoietin (EPO) which makes red blood cells in the bone marrow. In people with kidney failure, EPO levels drop, which causes a shortage of red blood cells and leads to anaemia. Treatment is usually injections of a synthetic version of EPO.

PRCA occurs when the EPO fails to increase the production of red blood cells. People with PRCA develop an antibody that stops the EPO from working properly, so the anaemia comes back.

It was previously believed that most cases of PRCA were due to poor handling and storage of EPO and numbers have dropped since strict guidelines have been put in place. However, once the antibody link was discovered, it was realised that the method of injection is important.

Antibodies are more likely to form when patients have injections under the skin (subcutaneously) rather than into a vein (intravenously). Haemodialysis patients are therefore usually given EPO intravenously to minimise the number of antibodies that might form.

People who self-administer EPO should follow the manufacturer’s guidelines very carefully and talk to their healthcare team if they have any questions over dosage or use.

How is PRCA diagnosed?

PRCA is diagnosed by a blood test for the antibody and by a bone marrow biopsy to check whether any new red blood cells are being produced.

Does PRCA affect other parts of the body?

PRCA is only known to affect the bone marrow.

Does PRCA run in families?

There is some recent research that PRCA may run in families, but the precise details of any inheritance patterns are still being investigated.

How is PRCA treated?

The first form of treatment for PRCA is usually stopping EPO injections as they no longer work and further usage may cause more problems.

New medications called hypoxia inducible factor prolyl hydroxylase (HIF-PH) inhibitors, such as roxadustat and daprodustat, may be prescribed to increase production of red blood cells and levels of haemoglobin in the blood. They are usually given as tablets and are only suitable for more advanced chronic kidney disease (stages 3 to 5) but where the patient has not yet started dialysis.

Blood transfusions are likely to be needed to treat anaemia.

Immunosuppressant medication may also be prescribed to control the antibodies.

In some cases, PRCA has been found to disappear after a kidney transplant. However, it is not currently known why this occurs and transplant is not a used treatment for PRCA itself.

Where can I get more information or support about PRCA?

We are not aware of any UK-based support groups for PRCA. If you know of any PRCA disease patient groups, please let us know so we can share their details.

Publication date: 11/2023

Review date: 11/2026

This resource was produced according to PIF TICK standards. PIF TICK is the UK’s only assessed quality mark for print and online health and care information. Kidney Care UK is PIF TICK accredited.