Pure red cell aplasia explained

Pure red cell aplasia (PRCA) or erythroblastopenia refers to a type of aplastic anemia affecting the precursors to red blood cells but usually not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells. There are multiple etiologies that can cause PRCA. The condition has been first described by Paul Kaznelson in 1922.^[1]

Signs and symptoms

Signs and symptoms may include:

• Pale appearance
• Rapid heart rate
• Fatigue

Causes

Causes of PRCA include:

Treatment

PRCA is considered an autoimmune disease as it will respond to immunosuppressant treatment such as cyclosporin in many patients,^[2] though this approach is not without risk.^[3]

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.^[4]

See also

• Diamond–Blackfan anemia (genetic red cell aplasia)
• Aplastic anemia (aplasia affecting other bone marrow cells as well)

Notes and References

1. Kaznelson . P . vanc. 1922 . Zur Entstehung der Blutplättchen . Verh Dtsch Ges Inn Med. . 34 . 557–8 .
2. Sawada K, Hirokawa M, Fujishima N, etal . 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 . 92 . 8 . 1021–8 . August 2007 . 17640861 . 10.3324/haematol.11192. free .
3. Sawada K, Fujishima N, Hirokawa M . Acquired pure red cell aplasia: updated review of treatment . Br. J. Haematol. . 142 . 4 . 505–14 . August 2008 . 18510682 . 2592349 . 10.1111/j.1365-2141.2008.07216.x.
4. National Organization for Rare Disorders. (2003). Nord Guide to Rare Disorders. Lippincott Williams & Wilkins.