Pure Red Cell Aplasia Workup

Updated: May 27, 2021
  • Author: Srikanth Nagalla, MD, MS, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Workup

Laboratory Studies

The following blood tests should be obtained in suspected pure red cell aplasia (PRCA):

  • Complete blood cell count (CBC) count
  • Red blood cell (RBC) indices
  • Reticulocyte count
  • White blood cell (WBC) differential analysis of WBCs

Other studies to consider include the following:

  • Iron studies, especially iron saturation and serum ferritin levels, are used to diagnose hemosiderosis; this possibility should be considered in patients who have received multiple transfusions.
  • Serum vitamin B-12 and folate levels might be indicated in patients with macrocytosis.
  • Lactate dehydrogenase (LDH), indirect bilirubin, and serum haptoglobin levels are used to detect hemolysis.
  • Hemoglobin A 2 and hemoglobin F are used to rule out thalassemia.
  • Flow cytometry is used to diagnose hematological malignancies and T-cell disorders.

Tests to identify infection, including the following, are indicated:

  • Parvovirus B19 infection [29]
  • Hepatitis
  • Infectious mononucleosis

Tests to detect autoimmune disorders should include the following:

  • Antinuclear antibody test
  • C-reactive protein (CRP) level
  • Erythrocyte sedimentation rate (ESR)
  • Quantitative immunoglobulin analysis
  • Direct Coombs test to detect an autoimmune hemolytic anemia
  • Tests for thyroiditis, diabetes mellitus, rheumatoid arthritis, Sjögren syndrome, and systemic lupus erythematosus (SLE) might be indicated.

In addition, the following tests are helpful in diagnosing Diamond-Blackfan syndrome:

  • Hemoglobin F assay
  • “I” antigen on the surface of erythrocytes
  • Adenosine deaminase determination

Peripheral smears demonstrate a normocytic anemia in most cases of PRCA. However, macrocytic anemia occurs in Diamond-Blackfan and Good syndromes and in HIV infections. Peripheral smears can be used to screen for infectious mononucleosis, megaloblastosis, and hematological malignancies.

Bone marrow histology

Bone marrow aspiration smears in PRCA usually reveal a normocellular marrow. An absence of erythroblasts is noted, whereas more immature erythrocyte progenitors are present (maturation arrest). WBCs and platelet maturation are normal. Bone marrow can be used to evaluate iron stores and help diagnose megaloblastosis and hematological malignancies.

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Imaging Studies

Uses of imaging studies include the following:

  • Positron emission tomography (PET) and computed tomography (CT) scans are used to detect thymomas.
  • Spleen size can be determined by ultrasound imaging.
  • Appropriate imaging studies are used to help diagnose and evaluate hematological malignancies.
  • Duel-energy x-ray absorptiometry scans are used to assess osteopenia and osteoporosis due to corticosteroid therapy.
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Procedures

Procedures can include the following:

  • Bone marrow aspiration and biopsy
  • Appropriate biopsies to diagnose hematological malignancies.
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