Managing anemia in pediatric office practice: Part 1.

Abstract

1. George B. Segel, MD* 2. Michael G. Hirsh, MD† 3. Stephen A. Feig, MD‡ 1. *Professor of Pediatrics and Chief of Hematology/Oncology 2. †Clinical Associate Professor of Pediatrics, Strong Children’s Hospital, University of Rochester, Rochester, NY 3. ‡Professor of Pediatrics and Chief of Hematology/Oncology, Mattel Children’s Hospital at UCLA, Los Angeles, CA After completing this article, readers should be able to: 1. Compare and contrast the pathogenesis of iron deficiency anemia in toddlers and older children. 2. Describe the genetic defects resulting in beta-thalassemia and hemoglobin E and differentiate between heterozygous and homozygous defects. 3. Outline the molecular basis for alpha-thalassemia syndromes, including silent carrier, alpha-thalassemia trait, hemoglobin H, and Bart’s hemoglobinopathy. 4. Describe the pathogenesis of spherocytes in herediatry spherocytosis and the reasons for the shortened red cell survival. 5. Characterize the risk of “aplastic crisis” in hereditary spherocytosis and the requisite monitoring and management. Children’s hematologic and oncologic problems often present initially to the pediatrician. This article provides direction for the diagnosis and office treatment of anemia and guidelines for follow-up and, where appropriate, referral to a subspecialist. Part 1 considers iron deficiency, beta- and alpha-thalassemia trait, and hereditary spherocytosis (Table 1⇓). Part 2 considers sickle cell syndromes, glucose-6-phosphate dehydrogenase (G6PD) deficiency, and transient erythroblastopenia. These topics were proposed by practicing pediatricians in our communities as those of particular concern in their office practices. View this table: Table 1. Red Blood Cell (RBC) Disorders The traditional measurements of hemoglobin concentration and hematocrit may not be sufficient to determine the presence of anemia of childhood. The “normal values” cited in clinical pathology laboratory reports are unlikely to apply to children. Tables of normal values for children of different ages have been published (Table 2⇓). In general, the hemoglobin is high at birth and falls during the initial 6 to 8 weeks of life to a physiologic nadir before it gradually increases to childhood levels. Patients who suffer from cyanotic congenital heart disease or chronic obstructive pulmonary disease may have values that are considerably higher than children who do not have these problems. These children, therefore, may be anemic when their hemoglobin and hematocrit values are within the normal range …