Acquired Pure Red Cell Aplasia: Causes and Treatment

Questions and Answers

What characterizes Acquired Pure Red Cell Aplasia (aPRCA)?

A decline in the production of red blood cells in the bone marrow

Which of the following is NOT a known cause of aPRCA?

Genetic mutations

Which drug used to treat arthritis has been associated with causing aPRCA?

Penicillin

What type of treatment for aPRCA involves drugs that aim to suppress the immune system?

Immunosuppressants Signup and view all the answers

What is the response rate of corticosteroids as monotherapy in treating aPRCA?

47% Signup and view all the answers

Which drug for aPRCA treatment has a response rate of 74% when used alone?

Cyclosporine A Signup and view all the answers

Study Notes

Acquired Pure Red Cell Aplasia

Acquired Pure Red Cell Aplasia (aPRCA) is a rare bone marrow disorder characterized by a decline in the production of red blood cells (erythrocytes) in the bone marrow. Affected individuals may experience fatigue, lethargy, and/or abnormal paleness of the skin (pallor). This disorder has several known causes, including autoimmune disorders, infections, and certain drugs.

Causes

aPRCA is considered an autoimmune disorder, and it can be caused by a tumor of the thymus gland (thymoma), certain drugs, or a viral infection. Specific drugs that have been known to cause aPRCA include sulfonylureas, penicillin, phenytoin, and phenobarbitol, which are used in treating arthritis, epilepsy, and other conditions.

Treatment

The treatment for aPRCA involves immunosuppression, which can include corticosteroids, cyclosporine A, and cyclophosphamide. These drugs aim to suppress the immune system and reduce the autoimmune response that is causing the destruction of red blood cells.

Corticosteroids as monotherapy, such as prednisone, have a response rate of 47% (95% confidence interval [CI], 34-60). Cyclosporine A, a calcineurin inhibitor, has a response rate of 74% (95% CI, 66-82), with similar response rates in first-line and second-line treatments, as well as when used as monotherapy. Cyclophosphamide, an alkylating agent, has a response rate of 49% (95% CI, 35-64), which is higher when combined with corticosteroids and used in second-line treatment. Sirolimus, a mammalian target of rapamycin (mTOR) inhibitor, has an response rate of 87% (95% CI, 68-100).

Conclusion

Acquired Pure Red Cell Aplasia is a rare bone marrow disorder that can be caused by autoimmune disorders, infections, and certain drugs. The treatment for this disorder involves immunosuppression, including corticosteroids, cyclosporine A, cyclophosphamide, and sirolimus. The response rate to these treatments varies, with corticosteroids having a response rate of 47%, cyclosporine A having a response rate of 74%, and cyclophosphamide having a response rate of 49%.