How to Approach Neutropenia in Childhood

Kelly Walkovich, MD 2. Laurence A. Boxer, MD 1. Department of Pediatrics, University of Michigan, Ann Arbor, MI. * Abbreviations: AIN: : autoimmune neutropenia AML: : acute myelogenous leukemia ANC: : absolute neutrophil count CBC: : complete blood cell count MDS: : myelodysplastic syndrome rhG-CSF: : recombinant human granulocyte colony-stimulating factor SCN: : severe congenital neutropenia SDS: : Shwachman-Diamond syndrome WBC: : white blood cell count 1. Patients presenting with recurrent fevers, mouth ulcers and gingivitis should be evaluated for neutropenia. 2. The use of recombinant human granulocyte colony-stimulating factor (rhG-CSF) in the management of cyclic neutropenia and severe congenital neutropenia has dramatically decreased clinical symptoms and has decreased mortality from infectious causes. After completing this article, readers should be able to: 1. Recognize patients who have concerning features of history, physical examination, or laboratory results that warrant further investigation for possible neutropenia or other immunodeficiency. 2. Define mild, moderate, and severe neutropenia. 3. Understand that neutropenia can arise from acquired or intrinsic conditions. Know which causes of neutropenia are most commonly encountered in childhood. 4. Recognize why disorders of neutrophil production and release from the bone marrow carry more risk for bacterial infection than peripheral neutropenia associated with normal bone marrow morphology. 5. Understand why neutropenic patients undergoing immunosuppressive therapy are more at risk for a serious bacterial infection than patients who have isolated neutropenia. 6. Understand the impact that treatment with recombinant human granulocyte colony-stimulating factor has had on the outcome of patients who have severe congenital and cyclic neutropenia. 7. Know when to refer to a pediatric hematologist/oncologist. A case study is provided to illustrate key aspects of the care of patients who have neutropenia. A 13-month-old boy presents to his primary care physician for his standard 1-year well-child check. He has had four episodes of otitis media since starting child care, all of which resolved with standard antibiotics. His mother reports that he has been …