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Description, Types, Causes, Diagnosis, Treatment
What is Pure red cell aplasia (PRCA)?
Pure red cell aplasia (PRCA) is a rare blood condition where the bone marrow fails to produce the required amount of red blood cells for normal activities. This results in chronic anaemia. PRCA can be acquired or inherited. The inherited form is known as Diamond Blackfan Anaemia and is usually diagnosed within the first two years of the baby’s life. The acquired form which is more common occurs in adulthood.
PRCA is distinguished from aplastic anaemia as it affects only red blood cell production with platelets and white blood cells remaining normal. In aplastic anaemia, all three are affected – red blood cells, white blood cells and platelets.
Pure red cell aplasia affects both men and women equally.
Inherited PRCA is due to a defective genetic mutation resulting in Diamond Blackfan Anaemia (DBA). Some of the identified genes in DBA include RPS19, RPL5, RPS10, RPL11, RPL35A, RPS7, RPS17, RPS24, RPS26 and GATA1.
The most common cause of acquired and idiopathic PRCA is thought to be an autoimmune mechanism where the body’s immune cells attack its own red blood cells. Some of the risk factors include:
- Underlying autoimmune disorders like rheumatoid arthritis or clonal large granular lymphocytes which attack the red cell lines in the bone marrow
- Viruses such as hepatitis, parovirus B19
- Drugs and toxic chemicals
- Shortness of breath
- Irregular and rapid heart beat
- Complete blood counts
- Reticulocyte counts
- Blood smear to be examined under a microscope
- Bone marrow biopsy using needle aspiration technique
- Blood transfusions – although this is not a cure for Pure red cell aplasia, it can treat the low red blood cell counts
- Iron chelation – Apart from regular blood transfusions, iron chelation therapy may be required to prevent iron toxicity from building up in the vital organs
- Immunosuppressive drug therapy is recommended if the underlying cause is an autoimmune factor. Drugs like cyclophosphamide, cyclosporine and azathioprine may be used. In some cases, rituximab may be effective
- PRCA caused by viruses such as parovirus B19 is usually treated with immunoglobulin infusions
As of today, bone marrow transplant is the only cure for Pure red cell aplasia. A full HLA match with a sibling or unrelated donor is recommended.