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Updated: Mar 27 2023

Pure Red Cell Aplasia


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  • Snapshot
    • An 11-month-old Caucasian boy presents to the emergency department for severe fatigue. His symptoms progressively worsened to the point where he is unresponsive. On physical exam, the patient has a flat nasal bridge and triphalangeal thumbs. Laboratory testing is significant for an isolated macrocytic anemia, reticulocytopenia, and increased HbF. Genetic testing is pursued, which demonstrates a ribosomopathy.
  • Introduction
    • Clinical definition
      • isolated anemia and reticulocytopenia due to a pure red cell aplasia (PRCA)
    • Etiology
      • may be due to congenital (e.g., Diamond-Blackfan anemia) or acquired (e.g., transient erythroblastopenia, paraneoplastic syndromes, and parvovirus B19 infection) causes
      • Pure Red Cell Aplasia
      • Cause
      • Clinical Findings
      • Laboratory Findings
      • Treatment
      • Diamond-Blackfan anemia (DBA)
      • Typically occurs in infancy
      • Congenital abnormalities
        • growth retardation
        • craniofacial abnormalities
          • e.g., hypertelorism and a flat nasal bridge
        • thumb abnormalities
          • e.g.,triphalangeal thumbs
      • Predisposition to cancer
        • acute myelogenous leukemia
        • myelodysplastic syndrome
      • Etiology
        • congenital impairments affecting ribosome synthesis
      • Macrocytic, normochromic, and non-megaloblastic anemia
      • Reticulocytopenia
      • Increased HbF
      • Corticosteroids and blood transfusions
      • Hematopoietic cell transplantation in patients unresponsive to steroids
      • Transient erythroblastopenia of childhood (TEC)
      • Transient/temporary red cell aplasiaDBA is chronic
      • The child is otherwise healthy
      • Etiology
        • unknown
      • Normocytic anemia at the time of diagnosis
      • Reticulocytopenia
      • Patients typically recover 1-2 months without treatment
        • RBC transfusions may be needed for disabling symptoms
      • Parvovirus B19
      • Patients with an underlying hemolytic process may experience an aplastic crisis with parvovirus B19 infection
        • e.g., sickle cell anemia, hereditary spherocytosis, and pyruvate kinase deficiency
      • Etiology
        • viral infection of erythroid progenitors via a P-antigen receptor resulting in lytic destruction of proerythroblasts
      • Proerythroblasts and absent erythroid precursors may be seen in the bone marrow
      • Otherwise healthy children do not require treatment
      • Patients with an underlying hemolytic process receive RBC transfusions for symptomatic anemia
      • Paraneoplastic syndrome
      • Hemoglobin decline in a patient who has not had a transfusion and previously responded to erythropoietinEtiologyunderlying malignancy can result in the production of anti-erythropoietin antibodiesmalignanciesthymomamyelodysplastic syndromes
      • Bone marrow aspirate
        • severe erythroid hypoplasia
        • very little RBC precursors
      • Present anti-erythropoietin antibodies
      • Transfusions for symptomatic anemia
      • Discontinue recombinant erythropoietin (EPO) products
      • Immunosuppression
    • Transient/temporary red cell aplasia
      • DBA is chronic
    • The child is otherwise healthy
    • Etiology
      • unknown
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