TABLE 4–1CLASSIFICATION OF PURE ERYTHROID APLASIA |Favorite Table|Download (.pdf) TABLE 4–1 CLASSIFICATION OF PURE ERYTHROID APLASIA
Fetal Red Cell Aplasia (non-immune hydrops fetalis, parvovirus 19 infection in utero)
Blackfan-Diamond disease (RPS19 mutations in 25% of cases)
Transient pure red cell aplasia
Transient erythroblastopenia of childhood
Acute parvovirus B19 infection in the setting of underlying hemolytic anemia
Chronic pure red cell aplasia
Red cell aplasia in association with another disease (see Table 4–3)
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.
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.
TABLE 4–2SOME DRUGS ASSOCIATED WITH THE DEVELOPMENT OF ERYTHROID APLASTIC CRISIS |Favorite Table|Download (.pdf) TABLE 4–2 SOME DRUGS ASSOCIATED WITH THE DEVELOPMENT OF ERYTHROID APLASTIC CRISIS
|Generic Name |
|Alpha-Methyldopa (Aldomet) |
|Sulfobromophthalein sodium (bromsulphthalein) |
|Lindane (gamma benzene hexachloride) |
|Valproic acid |
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.
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 ...
Pop-up div Successfully Displayed
This div only appears when the trigger link is hovered over.
Otherwise it is hidden from view.