Management of acquired pure red cell aplasia (PRCA)

Management should involve specialists in bone marrow disorders and may require hospital admission. Primary acquired PRCA is an idiopathic autoimmune disorder. Causes of secondary acquired PRCA include pregnancy, medications, parvovirus B19 (in chronically immunocompromised individuals), and hematologic malignancies. Pregnancy-associated PRCA often resolves with delivery and medication-induced PRCA typically resolves after drug discontinuation. Transfusions can be required until medication-induced PRCA resolves and can be a transitional strategy for other types of PRCA. Refer to UpToDate for details.

CBC: complete blood count; IVIG: intravenous immune globulin; PCR: polymerase chain reaction; PRCA: pure red cell aplasia.

* Diagnosis is made based on:

Normochromic, normocytic anemia
Absolute reticulocyte count <10,000/microL
Normal WBC and platelet counts
Normocellular bone marrow with <1% erythroblasts or proerythroblasts and basophilic erythroblasts <5% of nucleated cells
No other obvious cause of anemia

¶ May also include patients with symptomatic anemia who are not transfused.

Δ Decision-making requires expertise in bone marrow disorders and the patient's clinical status. Alternatives to cyclosporine and/or a glucocorticoid include sirolimus, methotrexate, oral cyclophosphamide, and enrollment in a clinical trial. Individuals with hematologic malignancy or monoclonal gammopathy may have a response to treatment for the malignancy or to plasma-cell directed therapy, respectively.

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Management of acquired pure red cell aplasia (PRCA)