SPLENIC FUNCTION AND HEMATOLOGIC CHANGES DURING THE FIRST YEAR OF LIFE IN SICKLE CELL ANEMIA

Abstract

Seven infants with sickle cell anemia have been identified in routine screening of cord blood from 1500 neonates. These children have been followed serially for up to 2 years. Hemoglobin levels fell most rapidly during the first 3 months and then declined more slowly, stabilizing between 7 and 10 gm% by 10 months of age. The post natal decline of Hgb F was slower than normal but showed considerable variability. There was close correlation between the rate of fall of Hgb F and the development and severity of hemolysis. Clinical vasocclusive symptoms occurred as early as 4 months but were also variable. 99mTc colloid scans showed normal splenic activity in all infants before 6 months. The onset of functional asplenia was documented in four infants at 7, 8, 10 and 11 months. Thus, functional asplenia is an acquired defect in sickle cell anemia. Howell-Jolly bodies were noted prior to the abnormal scan. Functional asplenia occurred when Hgb F fell below 20%. Two infants retain normal splenic function at 12 and 20 months. These observations indicate variability in the development of hematologic and splenic abnormalities in children with homozygous Hgb S disease. The fact that splenic dysfunction, with resultant susceptibility to overwhelming sepsis may occur as early as 7 months of age in this disease, provides impetus for early diagnosis.

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