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May 1964

Reticulocytopenia in Sickle Cell Disease: Aplastic Episodes in the Course of Sickle Cell Disease in Children

Author Affiliations

Evan Charney, MD, Family Health Care Program, Children's Hospital Medical Center, Boston, Mass 02115.; Associate Resident in Pediatrics, Strong Memorial Hospital (Dr. Charney); Associate Professor of Pediatrics (Dr. Miller).; From the Department of Pediatrics, University of Rochester School of Medicine and Dentistry and Strong Memorial Hospital.

Am J Dis Child. 1964;107(5):450-455. doi:10.1001/archpedi.1964.02080060452004

Temporary failure of red cell production has been well documented in hematologically normal persons, usually in association with infection, allergy, or a toxin.1,2 The brief duration of these episodes (seven to 14 days) in comparison to the relatively long life span of the normal erythrocyte (120 days) results in only a mild, transient, and usually unimportant drop in hemoglobin concentration. The same temporary failure in a patient with a congenital hemolytic anemia, on the other hand, is a dramatic event. In sickle cell disease, where the mean red cell life span has been estimated to range from 6 to 21.5 days 3,4 the same "aplastic" episode can result in a precipitous fall in hemoglobin to as low as half the steady state level over a two to seven day period. Thus a child who has maintained his hemoglobin at 6 gm% by a continuous brisk reticulocytosis may present with