Pure red cell aplasia

Signs and symptoms

Signs and symptoms may include:

• Pale appearance
• Rapid heart rate
• Fatigue

Causes

Causes of PRCA include:

• Autoimmune disease.
• Thymoma.
• Viral infections such as HIV, herpes, parvovirus B19 (Fifth disease), or hepatitis.
• Lymphoproliferative. Association of pure red cell aplasia with T-cell large granular lymphocyte leukemia is well recognized, especially in China.
• Idiopathic. Many cases of PRCA are considered idiopathic in that there is no discernible cause detected.
• Drugs such as mycophenolic acid or erythropoietin.
• Congenital. The term "hereditary pure red cell aplasia" has been used to refer to Diamond–Blackfan anemia.

Diagnosis

Treatment

PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as cyclosporin in many patients, though this approach is not without risk.

It has also been shown to respond to treatments with rituximab and tacrolimus.

For cases related to B19 parvovirus, administration of commercial immunoglobulin can treat or cure parvovirus by replacing neutralizing antibodies.

References

References

1. (1922). "Zur Entstehung der Blutplättchen". Verh Dtsch Ges Inn Med..
2. (January 2008). "Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group". Haematologica.
3. (December 2000). "Pure red cell aplasia caused by Parvovirus B19 infection in solid organ transplant recipients: a case report and review of literature". Clinical Transplantation.
4. (1998). "Association of pure red cell aplasia with T large granular lymphocyte leukaemia". J. Clin. Pathol..
5. (2008). "Three episodes of acquired pure red cell aplasia restricted to pregnancy.". Journal of Perinatal Medicine.
6. Petrochko C (2009). [http://www.medpagetoday.com/ProductAlert/Prescriptions/15545 "FDA Strengthens Warning on Transplant Drug."] ''Medpage Today.'' 14 August 2009. Accessed 19 August 2009.
7. (November 2007). "Epoetin-induced pure red cell aplasia: diagnosis and treatment.". Current Opinion in Nephrology and Hypertension.
8. {{OMIM. 105650
9. (August 2007). "Long-term outcome of patients with acquired primary idiopathic pure red cell aplasia receiving cyclosporine A. A nationwide cohort study in Japan for the PRCA Collaborative Study Group". Haematologica.
10. (August 2008). "Acquired pure red cell aplasia: updated review of treatment". Br. J. Haematol..
11. National Organization for Rare Disorders. (2003). Nord Guide to Rare Disorders. Lippincott Williams & Wilkins.