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August 1994

Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease

Author Affiliations

Cooperative Study of Sickle Cell Disease
From the Department of Pediatrics, State University of New York Health Science Center at Brooklyn (Drs Brown and Miller); the New England Research Institute, Watertown, Mass (Dr Sleeper); the Division of Pediatric Hematology, University of Miami (Fla) (Dr Pegelow); the Children's Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania Medical School, Philadelphia (Dr Gill); and the Biostatistics Research Branch, National Heart, Lung, and Blood Institute, Bethesda, Md (Dr Waclawiw). Clinical centers and senior investigators in the Cooperative Study of Sickle Cell Disease are listed later in this report.

Arch Pediatr Adolesc Med. 1994;148(8):796-804. doi:10.1001/archpedi.1994.02170080026005

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.

Research Design:  Prospective natural history study.

Setting:  Nineteen pediatric sickle cell centers across the United States.

Patients:  Six hundred ninety-four infants with sickle cell disease (sickle cell anemia, sickle cell–hemoglobin C disease, and sickle–β-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.

Measurements and 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 α-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 α-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.(Arch Pediatr Adolesc Med. 1994;148:796-804)