Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease.
OBJECTIVE
To examine hematologic changes from birth to 5 years of age and establish hematologic reference values for infants and children with sickle cell disease.


METHODS
Prospective natural history study.


METHODS
Nineteen pediatric sickle cell centers across the United States.


METHODS
Six hundred ninety-four infants with sickle cell disease (sickle cell anemia, sickle cell-hemoglobin C disease, and sickle-beta-thalassemia) who were enrolled in the Cooperative Study of Sickle Cell Disease at younger than 6 months of age. Median follow-up time through 5 years of age was 4.1 years.


RESULTS
We present longitudinal analyses of total hemoglobin concentration, percent fetal hemoglobin values, mean corpuscular volumes, total bilirubin concentration, and red blood cell (RBC), "pocked" RBC, white blood cell, platelet, and reticulocyte counts. Anemia was apparent by 10 weeks of life in infants with sickle cell anemia (SS infants). This anemia was associated with a rising reticulocyte count consistent with a hemolytic process. The reticulocyte count of SS infants increased steadily, exceeding 12% at 5 years of age. The fetal hemoglobin concentration of SS infants declined more slowly than that of infants with sickle cell hemoglobin C disease (SC infants). Pocked RBC counts rose sharply after 6 months of age, and by 1 year, 28% of SS infants had abnormal counts, above 3.5%, indicating poor splenic function. At 3 years of age, 78% of SS patients and 32% of SC patients had abnormal pocked RBC counts. The SS patients with concurrent alpha-thalassemia had, after 6 months of age and throughout early childhood, a slightly higher mean total hemoglobin concentration and lower mean pocked RBC and reticulocyte counts than SS patients without alpha-thalassemia. The hematologic profile of SC infants more closely resembled that of normal black infants, but there was mild anemia (10.5 g/dL) and slightly elevated mean values for reticulocytes (3%) and fetal hemoglobin (3%) during early childhood.
Brown
A K
AK
Department of Pediatrics, State University of New York Health Science Center at Brooklyn.
Sleeper
L A
LA
Miller
S T
ST
Pegelow
C H
CH
Gill
F M
FM
Waclawiw
M A
MA
D000367:Age Factors; D000704:Analysis of Variance; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000150:complications; D001663:Bilirubin / Q000097:blood; D002675:Child, Preschool; D004906:Erythrocyte Count; D004909:Erythrocyte Indices; D004913:Erythrocytes, Abnormal; D005260:Female; D005319:Fetal Hemoglobin / Q000032:analysis; D006445:Hemoglobin C Disease / Q000097:blood* / Q000150:complications; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007958:Leukocyte Count; D016015:Logistic Models; D008137:Longitudinal Studies; D008297:Male; D010976:Platelet Count; D012016:Reference Values; D012156:Reticulocytes; D017086:beta-Thalassemia / Q000097:blood* / Q000150:complications
D006454:Hemoglobins; D005319:Fetal Hemoglobin; D001663:Bilirubin