Managing anemia in a pediatric office practice: Part 2.

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 Medical Center, 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. Recognize the signs and symptoms of sickle cell vaso-occlusive crisis. 2. Explain the factors contributing to the pathogenesis of sickle cell vaso-occlusion. 3. Delineate the reasons for the heightened susceptibility to infection in sickle cell disease. 4. Describe the biochemical basis for hemolysis in glucose-6-phosphate dehydrogenase deficiency. 5. Compare and contrast Diamond-Blackfan anemia and transient erythroblastopenia of childhood. Children’s hematologic and oncologic problems often present initially to the pediatrician. This article provides guidance for the diagnosis and office treatment of anemia and guidelines for follow-up and, where appropriate, referral to a subspecialist. Last month, part 1 considered iron deficiency, beta- and alpha-thalassemia trait, and hereditary spherocytosis. This month, part 2 considers sickle cell syndromes, glucose-6-phosphate dehydrogenase (G6PD) deficiency, and transient erythroblastopenia (Table 1⇓). These topics were proposed by practicing pediatricians in our communites as those of particular concern in their office practices. View this table: Table 1. Red Blood Cell (RBC) Disorders ### History and Physical Examination The severe sickle syndromes are most common among people of African or mixed African and Hispanic descent. Many states have newborn screening programs that detect hemoglobinopathies at birth. Beta-chain abnormalities, such as hemoglobins S, C, E, and beta-thalassemia, do not cause symptoms until the change from gamma-chain (fetal hemoglobin, alpha2 gamma2) production to beta-chain (alpha2beta2) production is nearly complete between the ages of 6 and 12 months. Therefore, children who have severe sickle syndromes and are homozygous for hemoglobin S or who are double heterozygous for hemoglobin S and other hemoglobins such as C, D, O-Arab, or beta-thalassemia usually develop symptoms during the second half of the first year of life. Symptoms of sickle cell diseases may result from the occlusion of blood vessels by sickling, from the anemia itself, or from a heightened susceptibility to infection. The vaso-occlusive symptoms can arise …