Pure red cell aplasia
The idiopathic form is the most common. Acquired secondary PRCA is uncommon. Diamond-Blackfan syndrome (hereditary PRCA) is rare. The occurrence of transient and reversible PRCA secondary to medications and infections is probably underreported.
Infectious etiologies include human immunodeficiency virus, respiratory tract infection, gastroenteritis, primary atypical pneumonia, Epstein-Barr virus, mumps, and viral hepatitis. In the majority of cases, acute transient PRCA is caused by parvovirus B19 infection. Medications implicated in PRCA include antiepileptic medications, sulfonamides, mycophenolate, azathioprine, chloramphenicol, thiamphenicol, isoniazid, procainamide, and clopidogrel. Although thymoma was once thought to be the main cause of acquired PRCA, new research has shown that thymomas may only be responsible for a minority of cases.
Prognosis varies depending on the type of PRCA and the individual disease course. Treatment is case dependent. Common treatments include transfusions, immunoglobulin therapy, corticosteroids, stem cell transplantation, plasmapheresis, lymphocytapheresis, and iron chelation. When associated with thymoma, surgical removal may be curative.
D61.01 – Constitutional (pure) red blood cell aplasia
50715003 – Pure red cell aplasia
- Anemia from iron or other nutritional deficiency such as vitamin B12 or folate, blood loss, chronic disease, hemolysis, or marrow failure such as myelodysplastic syndrome
- Autoimmune hemolytic anemia from any cause
- Drug-induced anemia
- Viral-mediated anemia from parvovirus B19, human immunodeficiency virus, Epstein-Barr virus, cytomegalovirus