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Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease.
Arch Pediatr Adolesc Med 1994; 148(8):796-804AP

Abstract

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-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.

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 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.

Authors+Show Affiliations

Department of Pediatrics, State University of New York Health Science Center at Brooklyn.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Multicenter Study
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

7519102

Citation

Brown, A K., et al. "Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease. Cooperative Study of Sickle Cell Disease." Archives of Pediatrics & Adolescent Medicine, vol. 148, no. 8, 1994, pp. 796-804.
Brown AK, Sleeper LA, Miller ST, et al. Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med. 1994;148(8):796-804.
Brown, A. K., Sleeper, L. A., Miller, S. T., Pegelow, C. H., Gill, F. M., & Waclawiw, M. A. (1994). Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. Archives of Pediatrics & Adolescent Medicine, 148(8), pp. 796-804.
Brown AK, et al. Reference Values and Hematologic Changes From Birth to 5 Years in Patients With Sickle Cell Disease. Cooperative Study of Sickle Cell Disease. Arch Pediatr Adolesc Med. 1994;148(8):796-804. PubMed PMID: 7519102.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Reference values and hematologic changes from birth to 5 years in patients with sickle cell disease. Cooperative Study of Sickle Cell Disease. AU - Brown,A K, AU - Sleeper,L A, AU - Miller,S T, AU - Pegelow,C H, AU - Gill,F M, AU - Waclawiw,M A, PY - 1994/8/1/pubmed PY - 1994/8/1/medline PY - 1994/8/1/entrez SP - 796 EP - 804 JF - Archives of pediatrics & adolescent medicine JO - Arch Pediatr Adolesc Med VL - 148 IS - 8 N2 - 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-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. 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 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. SN - 1072-4710 UR - https://www.unboundmedicine.com/medline/citation/7519102/Reference_values_and_hematologic_changes_from_birth_to_5_years_in_patients_with_sickle_cell_disease__Cooperative_Study_of_Sickle_Cell_Disease_ L2 - https://jamanetwork.com/journals/jamapediatrics/fullarticle/vol/148/pg/796 DB - PRIME DP - Unbound Medicine ER -