3A6Z/D64.9 Pure red cell aplasia, unspecified

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Aetiology

Pure red cell aplasia (PRCA) is an acquired, immune-mediated disorder that results in a decrease in the number of circulating red blood cells, leading to anemia. The exact cause of PRCA is unknown, however it is believed to be the result of a disordered immune system targeting and destroying red blood cells.

Diagnosis

PRCA is typically diagnosed using a blood test to measure the levels of red blood cells in the body. A low level of red cells, as well as low hemoglobin and hematocrit levels, are indicative of PRCA. A bone marrow biopsy may also be required to confirm the diagnosis.

Differential diagnosis

PRCA can be difficult to diagnose and differentiate from other forms of anemia, such as iron deficiency anemia, aplastic anemia, pernicious anemia, or vitamin B12 deficiency anemia.

Treatment

Treatment for PRCA is typically centered around suppressing the immune system, which is believed to be responsible for the destruction of the red blood cells. Corticosteroids are the primary treatment, with other immunosuppressive medications such as cyclosporine and azathioprine being used in some cases. In severe cases, plasma exchange or bone marrow transplantation may be necessary.

Prognosis

The prognosis of PRCA is generally good and most patients respond well to treatment. However, in some cases the condition may recur or progress to aplastic anemia, which is a more serious and potentially life-threatening condition.

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