Pure Red Cell Aplasia (PRCA)
What is pure red cell aplasia (PRCA)?
Pure red cell aplasia (PRCA) is a rare disorder of blood production in which the bone marrow, the spongy tissue in the center of the bones, fails to function in an adequate manner resulting in anemia. Red blood cells are responsible for carrying oxygen to the entire body.
The anemia can be either acquired, genetic (inherited) or induced by drugs. In many cases the cause is unknown (idiopathic). The disorder equally affects males and females.
The very rare genetic form, Diamond-Blackfan anemia, is usually diagnosed before the age of 1. About 25 percent of patients with Diamond-Blackfan anemia will have abnormal features in the face, head and hands. Children who are affected tend to be short for their age and puberty may be delayed.
What are the symptoms of acquired red cell aplasia (PRCA)?
The signs are typical of those in patients with anemia:
What causes acquired pure red cell aplasia (PRCA)?
Acquired PRCA is an uncommon autoimmune disorder that can have multiple causes. These may include:
- A parvovirus B19 infection, which may be toxic to the red blood cells starters (or precursors)
- Certain lymphocytic leukemias (blood cancers) such as large granular lymphocytic leukemia (LGLL)
- Thymoma (a tumor of the thymus, which is a lymphoid organ)
- Autoimmune diseases such as rheumatoid arthritis or lupus
- Common variable immunodeficiency (CVID)
In the case of drug-induced red cell aplasia, antibodies can occasionally develop to a drug used to treat patients undergoing dialysis. Other drugs may be toxic to the red blood cell precursors.