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DEFINITION

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  • Pure red cell aplasia describes anemia caused by an isolated depletion of erythroblasts characterized by severe reticulocytopenia and absent or markedly diminished marrow erythroid progenitors (erythroblasts).

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CLASSIFICATION

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Table Graphic Jump Location
TABLE 4–1CLASSIFICATION OF PURE ERYTHROID APLASIA
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ACUTE RED CELL APLASIA

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  • May result from transient marrow erythroblastopenia, which occurs in both children and adults. It is most common in the first several years of life.

  • Seen most often in patients with a hemolytic disorder, such as hereditary spherocytosis or sickle cell anemia, when a transient severe reduction in erythropoiesis causes a rapid fall in hemoglobin level—called an (erythroid) aplastic crisis.

  • May also be seen in patients who are hematologically normal.

  • True prevalence is unknown, and it is assumed that many mild cases are not detected.

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Etiology

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  • Most patients with aplastic crises are infected with B19 parvovirus, but occasionally another viral infection may be responsible.

  • IgG inhibitors of erythroid colony formation in vitro have been found in some patients with a condition called transient erythroblastopenia of childhood.

  • Drugs may induce aplastic crises, either by an immunologic mechanism or by direct toxicity. Commonly implicated drugs are listed in Table 4–2.

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Table Graphic Jump Location
TABLE 4–2SOME DRUGS ASSOCIATED WITH THE DEVELOPMENT OF ERYTHROID APLASTIC CRISIS
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Clinical Features

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  • Frequently, the patient has had a recent febrile illness, often with upper respiratory symptoms, gastrointestinal symptoms, or headache.

  • Listlessness, increasing pallor, and tachycardia are characteristic.

  • Usually no other significant changes are found on physical examination.

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Laboratory Features

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  • Evidence of an underlying hematologic disorder, such as hereditary spherocytosis or sickle cell anemia, may be present.

  • Anemia and reticulocytopenia are characteristic. They are often severe.

  • Granulocyte and platelet counts are usually normal.

  • Erythroid cells are depleted in the marrow early in the illness, but reappear just before recovery; thus, if the marrow is tested during recovery, the erythroblastopenia may be missed.

  • Reticulocytosis is the first sign of recovery, and some nucleated red cells ...

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