Brown A K, Sleeper L A, Miller S T, Pegelow C H, Gill F M, Waclawiw M A
Department of Pediatrics, State University of New York Health Science Center at Brooklyn.
Arch Pediatr Adolesc Med. 1994 Aug;148(8):796-804. doi: 10.1001/archpedi.1994.02170080026005.
To examine hematologic changes from birth to 5 years of age and establish hematologic reference values for infants and children with sickle cell disease.
Prospective natural history study.
Nineteen pediatric sickle cell centers across the United States.
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.
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.
研究从出生到5岁的血液学变化，并为镰状细胞病患儿建立血液学参考值。
前瞻性自然史研究。
美国19个儿科镰状细胞中心。
694例镰状细胞病婴儿（镰状细胞贫血、镰状细胞 - 血红蛋白C病和镰状 - β地中海贫血），这些婴儿在6个月龄前参加了镰状细胞病合作研究。至5岁的中位随访时间为4.1年。
我们对总血红蛋白浓度、胎儿血红蛋白百分比值、平均红细胞体积、总胆红素浓度以及红细胞（RBC）、“有凹痕”红细胞、白细胞、血小板和网织红细胞计数进行了纵向分析。镰状细胞贫血（SS型婴儿）患儿在出生后10周时出现明显贫血。这种贫血与网织红细胞计数升高有关，符合溶血过程。SS型婴儿的网织红细胞计数稳步上升，5岁时超过12%。SS型婴儿的胎儿血红蛋白浓度下降速度比镰状细胞 - 血红蛋白C病婴儿（SC型婴儿）慢。6个月龄后“有凹痕”红细胞计数急剧上升，到1岁时，28%的SS型婴儿计数异常，高于3.5%，表明脾功能不佳。3岁时，78%的SS型患者和32%的SC型患者“有凹痕”红细胞计数异常。同时患有α地中海贫血的SS型患者在6个月龄后及整个幼儿期，其平均总血红蛋白浓度略高，平均“有凹痕”红细胞和网织红细胞计数低于无α地中海贫血的SS型患者。SC型婴儿的血液学特征更类似于正常黑人婴儿，但在幼儿期有轻度贫血（10.5 g/dL），网织红细胞（3%）和胎儿血红蛋白（3%）的平均值略有升高。
