Approach Considerations

The initial treatment plan should include transfusions for patients who are severely anemic and have cardiorespiratory failure. Anemia is more severe in patients with pure red cell aplasia (PRCA) who have ongoing hemolysis (aplastic crises).

Medications that could cause PRCA should be discontinued.

Children with PRCA should be observed and not aggressively treated to avoid corticosteroid-related growth retardation. This caution is feasible since PRCA in children is often transient and reversible. However, transfusion should be administered if indicated.

Infections should be treated. High-dose intravenous immunoglobin therapy should be considered for parvovirus B19 infections.^[30]PRCA due to medication or infections is usually reversible within a few months, if not earlier. However, immunotherapy may be needed to reverse erythropoiesis-stimulating agent (ESA)–related PRCA.

Underlying conditions should be treated. These conditions include a thymoma, hematological malignancies such as T-cell large granular lymphocyte leukemia,^[31]solid tumors, and systemic lupus erythematosus (SLE). Surgery or gamma irradiation of the thymus should be considered in a patient with a thymoma.

PRCA considered to be idiopathic and due to autoimmunity should be initially treated with corticosteroids.^{[1, 2, 27]}A response is expected within 4-6 weeks in about 45% of patients. Corticosteroids should be judiciously given to children to avoid growth retardation. Immunosuppressive agents have an important role. Immunosuppressive agents used in PRCA include cyclophosphamide, 6-mercaptopurine, azathioprine, and cyclosporine A. Rituximab has been reported to be effective in managing PRCA.^{[32, 33, 28, 34]}Antithymic globulin (ATG) is another therapeutic option. Danazol has been helpful in some cases but is contraindicated in children. Plasmapheresis has been used to remove autoantibodies.

There are a number of reports of successful control of refractory/relapsed PRCA with sirolimus. However, multi-center and large-scale randomized controlled trials are needed to establish the efficacy of this treatment.^{[35, 36, 37, 38]}

Several patients have responded to plasmapheresis or lymphocytapheresis.^[39]

Autologous and nonmyeloablative allogeneic peripheral stem cell transplantation have been used, especially in patients whose disease is refractory to therapy.^[40]

Iron chelation should be considered in patients who have had multiple transfusions and have evidence of iron overload.

Surgical Care

Thymectomy might be indicated in patients with a thymoma. However, the procedure should not be performed in patients with a normal-sized thymus. About 30% of patients with thymomas respond to thymectomy.

Although not effective in most cases, splenectomy might be helpful in refractory cases. Splenectomy is indicated to manage pure red cell aplasia (PRCA) complicated by hypersplenism.

Consultations

Consultations with a hematologist and rheumatologist are indicated. A hematologist should be consulted for the diagnosis and management of pure red cell aplasia (PRCA). A rheumatologist should be consulted in patients with autoimmune disorders.

Activity

Activity might be limited in anemic patients.

Medication

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