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- Kelly Walkovich, MD
- Laurence A. Boxer, MD
- Department of Pediatrics, University of Michigan, Ann Arbor, MI.
Author Disclosure
Dr Walkovich has disclosed no financial relationships relevant to this article. Dr. Boxer has disclosed that he owns stocks/bonds in Amgen Inc.; serves as a consultant and on the speaker bureau of Alexion Pharmaceuticals; and is a consultant for Vidara Therapeutics. This commentary does not contain discussion of unapproved/investigative use of a commercial product/device.
- 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
Practice Gaps
Patients presenting with recurrent fevers, mouth ulcers and gingivitis should be evaluated for neutropenia.
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.
Objectives
After completing this article, readers should be able to:
Recognize patients who have concerning features of history, physical examination, or laboratory results that warrant further investigation for possible neutropenia or other immunodeficiency.
Define mild, moderate, and severe neutropenia.
Understand that neutropenia can arise from acquired or intrinsic conditions. Know which causes of neutropenia are most commonly encountered in childhood.
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.
Understand why neutropenic patients undergoing immunosuppressive therapy are more at risk for a serious bacterial infection than patients who have isolated neutropenia.
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.
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.
Case
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 …
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