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Pure red cell aplasia

ISSN 2398-2942

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Introduction

  • Selective failure of erythropoiesis (maturation arrest at early normoblast stage) but normal production of white blood cells and platelets.
  • Insidious onset, non-regenerative anemia.
  • Cause: potentially immune-mediated.
  • Signs: weight loss.
  • Diagnosis: bone marrow aspirate.
  • Treatment: immunosuppressive therapy may, on occasion, be helpful.
  • Prognosis: good to fair.

Presenting signs

  • Exercise intolerance.
  • Depression.
  • Pica
  • Anorexia.
  • Weight loss.

Acute presentation

  • Collapse, eg following exertion, due to anemia.

Pathogenesis

Etiology

  • Idiopathic.
  • Immune-mediated is possible.
  • May be secondary to parvovirus infection - suggested but not proven.

Pathophysiology

  • Serum immunoglobulin G inhibitor → decreased erythroid colony precursors → insidious onset anemia → physiological compensation → relatively mild signs even if severe anemia.

Timecourse

  • Gradual onset over weeks or months.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers