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Pure Red Cell Aplasia Treatment & Management

  • Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD  more...
 
Updated: Feb 25, 2014
 

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.[27] 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,[28] 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, 24] 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.[29, 30, 25, 31] 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.

Autologous and nonmyeloablative allogeneic peripheral stem cell transplantation have been used, especially in patients refractory to therapy.[32]

Several patients have responded to plasmapheresis or lymphocytapheresis.[33]

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

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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.

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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.

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Activity

Activity might be limited in anemic patients.

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Follow-Up

Further inpatient care and outpatient care

The goals of inpatient care are to diagnose pure red cell aplasia (PRCA) and any underlying disorders, initiate appropriate management, monitor the response to therapy, and manage the adverse effects of therapy.

Deterrence/prevention

Avoid medications that might cause pure red cell aplasia (PRCA).

Complications

Severe uncompensated anemia can cause myocardial damage and organ failure.

Multiple transfusions can cause hemosiderosis.

Corticosteroid therapy can cause osteopenia, osteoporosis, and growth retardation.

Patients who are on immunosuppressive therapy can develop an acute leukemia and aplastic anemia.

The possibility exists for the transmission of infections by transfusion therapy, intravenous immunoglobulin G, and antilymphocytic serum.

Patient Education

The consequences of iron overload due to multiple transfusions should be explained.

The possibility of the transmission of infections by transfusion therapy, intravenous immunoglobulin G, and antilymphocytic serum should be explained.

The adverse effects of corticosteroids, immunotherapy, and other aspects of management should be explained.

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Contributor Information and Disclosures
Author

Paul Schick, MD Emeritus Professor, Department of Internal Medicine, Jefferson Medical College of Thomas Jefferson University; Research Professor, Department of Internal Medicine, Drexel University College of Medicine; Adjunct Professor of Medicine, Lankenau Hospital

Paul Schick, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

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