Pure red cell aplasia (primary acquired)
Evidence: Antibody
Names
Acquired pure red cell aplasia
Adult pure red cell aplasia
Idiopathic pure red cell aplasia
Pure red cell aplasia
Primary acquired PRCA
PRCA
Description
Pure red cell aplasia (PRCA) occurs when red blood cells fail to fully develop after they are produced in the bone marrow. This leads to symptoms of anemia, including tiredness and pale skin. There are 2 types of PRCA caused by autoimmune processes:
Acquired primary PRCA typically affects adults and leads to symptoms of anemia. The trigger for primary PRCA is unknown. Acquired primary PRCA is caused by the immune system attacking developing red blood cells, preventing them from maturing into functional adult cells.
Acquired secondary PRCA, which is also autoimmune, is similar to acquired primary PRCA but the trigger is an infection, autoimmune disorder, pregnancy, or cancer. Drugs may also trigger secondary PRCA.
Patients may experience symptoms of anemia as well as symptoms of the underlying health condition. Treatments for both primary and secondary PRCA are available, but outcomes vary, and life expectancy is often markedly reduced.
Note: A third type of PRCA, Diamond-Blackfan syndrome, is due to genetic mutation and presents during infancy. It is not autoimmune.
Patient Groups
Bloggers
None found.
Prevalence
Primary pure red cell aplasia is very rare. No prevalence studies have been found.
Typical Age of onset
No correlation with specific ages is has been observed. However, autoimmune forms of PRCA are typically found in adults, whereas the non-autoimmune genetic form, Diamond-Blackfan PRCA, is present from birth.
Symptoms
Fatigue
Pale skin
Dizziness
Shortness of breath
Unexplained or easy bruising
