Canterbury DHB

Context

Pure Red Cell Aplasia

In This Section

Introduction and Key Points

Classification

Diagnosis

Management

Introduction and Key Points

Pure Red Cell Aplasia is a rare condition that occurs in congenital and acquired forms. The latter may be transient or chronic.

Key Points

For review of PRCA, see UpToDate.

Classification

Inherited: Diamond Blackfan Syndrome and other conditions.

Acquired:

Diagnosis

Management

Transient erythroblastopenia of childhood usually occurs between the ages of 6 months and 3 years. Parvovirus B19 binds to the P antigen on the red cell surface, and IgG antibodies directed against erythroblasts can be detected. Treatment is supportive, although blood transfusion may be needed, particularly if the patient has an inherited haemolytic anaemia.

Chronic acquired PRCA is an autoimmune disorder which often responds to immunosuppression. Blood transfusion again may be required. Erythropoeitin is not helpful. Thymectomy may be effective. Steroids, ATG, and cyclosporin may all be successful, but cytotoxic therapy is usually without benefit. Rituximab may produce prompt improvement (Karra K, Leuk Res 2006, 30). Up to half of acquired PRCA patients show some response to treatment.

Note: Following an ABO mismatched allogeneic stem cell transplant, about 20% of patients will have a significant period of red cell aplasia due to the recipients' anti A or B production.

About this Canterbury DHB document (4939):

Document Owner:

Mark Smith (see Who's Who)

Issue Date:

August 2017

Next Review:

August 2019

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 4939