Pure Red Cell Aplasia Clinical Presentation

Updated: Jan 06, 2017
  • Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Presentation

History

Presenting symptoms depend on the severity of the anemia. Some patients are virtually asymptomatic, whereas others have an uncompensated anemia, have cardiopulmonary distress, and are transfusion dependent. [1, 2, 26]

Patients with aplastic anemia, as opposed to pure red cell aplasia (PRCA), may have a history of bruising due to thrombocytopenia. [27]

Obtaining the history of medications that patients are taking is important. A history of recent infections, such as infectious mononucleosis or viral hepatitis, is important.

Patients who have an underlying hemolytic anemia can become markedly anemic if they develop PRCA. This is known as an aplastic crises and is caused by hemolysis that is ongoing while erythrocyte production is impaired. The possibility of an aplastic crisis should be considered in patients with a hemolytic anemia if reticulocyte counts are low and if they have had recent infections. In contrast, the development of anemia in PRCA in patients without hemolysis is often gradual and self-limited and, hence, not noticed.

To determine whether the patient has a secondary PRCA, ask about the possibility of pregnancy, signs of systemic lupus erythematosus (SLE), signs of a hematological malignancy, and signs of other possible disorders that can cause PRCA. A history of miscarriages might suggest SLE.

A history of autoimmune disorders such as type 1 diabetes, thyroiditis, and rheumatoid arthritis should be elicited. Dryness of eyes and mouth occurs in Sjögren syndrome.

Recognize that chronic renal failure and erythropoietin therapy, AB0-incompatible transfusion, and stem cell transplantation are associated with PRCA.

Diamond-Blackfan syndrome should be considered in a child with PRCA, retarded growth, and developmental defects.

Next:

Physical Examination

The severity of anemia and degree of compensation and cardiopulmonary distress should be assessed.

Evidence of bruising and mucocutaneous bleeding might suggest pancytopenia and aplastic anemia.

Evidence of recent infection such as a rash, nasal congestion, cough, and jaundice should be evaluated. Enlarged parotid glands might suggest mumps

Lymphadenopathy and splenomegaly may indicate the presence of an underlying lymphoproliferative disorder or infectious mononucleosis.

Evidence of an autoimmune disorder such as arthritis, type 1 diabetes, autoimmune hemolytic anemia, or thyroiditis should be assessed. Leg ulcers and splenomegaly can occur in hemolytic anemias.

A malar butterfly rash and arthritis suggests SLE.

Thymomas are rarely large enough to be detected during the physical examination.

Diamond-Blackfan syndrome is suggested by retarded growth and congenital abnormalities.

One should evaluate patients for complications of therapy such as hemosiderosis due to transfusion therapy, diabetes, and osteopenia and osteoporosis due to corticosteroid therapy, myelogenous acute leukemia due to immunotherapy, and infections due to blood product transfusions.

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