Podcast
Questions and Answers
What characterizes Acquired Pure Red Cell Aplasia (aPRCA)?
What characterizes Acquired Pure Red Cell Aplasia (aPRCA)?
Which of the following is NOT a known cause of aPRCA?
Which of the following is NOT a known cause of aPRCA?
Which drug used to treat arthritis has been associated with causing aPRCA?
Which drug used to treat arthritis has been associated with causing aPRCA?
What type of treatment for aPRCA involves drugs that aim to suppress the immune system?
What type of treatment for aPRCA involves drugs that aim to suppress the immune system?
Signup and view all the answers
What is the response rate of corticosteroids as monotherapy in treating aPRCA?
What is the response rate of corticosteroids as monotherapy in treating aPRCA?
Signup and view all the answers
Which drug for aPRCA treatment has a response rate of 74% when used alone?
Which drug for aPRCA treatment has a response rate of 74% when used alone?
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%.
Studying That Suits You
Use AI to generate personalized quizzes and flashcards to suit your learning preferences.
Description
Learn about the causes, symptoms, and treatment of Acquired Pure Red Cell Aplasia (aPRCA), a rare bone marrow disorder characterized by a decline in red blood cell production. Understand the role of immunosuppressive drugs like corticosteroids, cyclosporine A, cyclophosphamide, and sirolimus in managing aPRCA.
