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What is Pure red cell aplasia (PRCA)

Pure red cell aplasia (PRCA) is an uncommon disorder in which maturation (growth) arrest occurs in the formation of erythrocytes (red blood cells). Secondary PRCA occurs in patients with conditions such as autoimmune disorders, thymomas, systemic lupus erythematosus, hematologic malignancies, and solid tumors. The life expectancy of patients with idiopathic PRCA is about 1-2 decades. The survival of patients with congenital PRCA is limited. The lifespan of patients with secondary PRCA depends on the course of the underlying disorder.

Pure red cell aplasia (PRCA) is a rare condition that affects the bone marrow. Bone marrow contains stem cells which develop into the red blood cells that carry oxygen through the body, the white blood cells that fight infections, and the platelets that help with blood clotting. In people with PRCA, the bone marrow makes a reduced number of red blood cells (called anemia). As a result, affected people may experience fatigue, lethargy, and pale skin.[1][2] PRCA has many different causes. A rare congenital form of PRCA, called Diamond Blackfan syndrome, is an inherited condition that is also associated with other physical abnormalities. PRCA can also be due to certain medications, infections, pregnancy, renal failure, and conditions such as thymomas, autoimmune disease (such as systemic lupus erythematosus), cancers of the blood, and solid tumors. In many cases, the cause of the condition is unknown (idiopathic).[1][3] The treatment of PRCA aims to address the underlying cause of the condition and relieve the associated signs and symptoms.[1]

This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).

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