Pure Red Cell Aplasia Clinical Presentation

  • Author: Paul Schick, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 10, 2012
 

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, 19]

Patients with aplastic anemia, as opposed to pure red cell aplasia (PRCA), may have a history of bruising due to thrombocytopenia.[20]

Query patients about what medications they are taking.

Ask patients about a history of recent infections, such as infectious mononucleosis or viral hepatitis.

The possibility of an aplastic crisis should be considered in patients with a hemolytic anemia. This crisis occurs because hemolysis is ongoing while erythrocytes production is impaired.

In contrast, the development of anemia in PRCA in patients without hemolysis is gradual and self-limited. If PRCA is transient, the anemia is often 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 can occur in 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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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 Heart Association, American Society of Hematology, International Society on Thrombosis and Haemostasis, and New York Academy of Sciences

Disclosure: Nothing to disclose.

Specialty Editor Board

Rodger L Bick†, MD, PhD, FACP  Former Clinical Professor of Medicine, University of Texas Southwestern Medical Center; Former Director, Dallas and Pacific Thrombosis Hemostasis and Vascular Medicine Clinical Center

Rodger L Bick†, MD, PhD, FACP is a member of the following medical societies: American Association for Cancer Research, American Association for the Advancement of Science, American Association of Blood Banks, American Cancer Society, American College of Angiology, American College of Physicians, American Geriatrics Society, American Heart Association, American Medical Association, American Society for Clinical Pathology, American Society of Hematology, Association of Clinical Scientists, California Medical Association, California Thoracic Society, International College of Angiology, International Society of Hematology, International Society on Thrombosis and Haemostasis, New York Academy of Sciences, and Southwest Oncology Group

Disclosure: Nothing to disclose.

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

Disclosure: Medscape Salary Employment

Troy H Guthrie, Jr, MD  Director of Cancer Institute, Baptist Medical Center

Troy H Guthrie, Jr, MD is a member of the following medical societies: American Federation for Medical Research, American Medical Association, American Society of Hematology, Florida Medical Association, Medical Association of Georgia, and Southern Medical Association

Disclosure: Nothing to disclose.

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, 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 College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

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