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January 1960

Sickle-Cell Disease: The "Aplastic Crisis" and Erythroid Maturation Defect Occurring Simultaneously in Three Members of a Family

Author Affiliations

Richmond, Va.

A. D. Williams Research Fellow, Department of Hematology and Laboratory for Clinical Investigation, Medical College of Virginia. 100/76

AMA Arch Intern Med. 1960;105(1):76-82. doi:10.1001/archinte.1960.00270130092011

It is now recognized that bone-marrow failure rather than hyperhemolysis underlies the crises in sickle-cell disease.1,2 Bonemarrow failure has been reported in Cooley's hemolytic anemia,3 hereditary spherocytosis,4 and paroxysmal nocturnal hemoglobinuria.5 Maturation arrest of the neutrophil polymorphonuclear leukocytes has been noted in sickle-cell disease.6 Infection has been thought to precipitate bone-marrow aplasia in sickle-cell disease1,2 and in hereditary spherocytosis.7 The occurrence of "aplastic crisis" in several members of a family has been recorded in hereditary spherocytosis 7 and in sickle-cell disease.17 Erythroblastopenia has been noted during the course of viral pneumonia in patients without hemolytic blood diseases,8 and the same communication records eosinophilia in patients who had erythrocytic aplasia after exposure to allergens.

The association of chronic hemolytic anemias with megaloblastic arrest of the bone marrow has been documented. Some of these responded to vitamin B12 therapy,9 some were associated with pregnancy,10 and in others dietary deficiency of folie acid

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