Pediatric Autoimmune and Chronic Benign Neutropenia Treatment & Management

  • Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA   more...
 
Updated: Mar 29, 2011
 

Approach Considerations

Focus the treatment of autoimmune neutropenia on intercurrent infections. In patients with frequent infections, prophylactic antibiotics with trimethoprim and sulfamethoxazole may help, although no clinical trials have demonstrated a benefit.

If the patient is free from significant infection, treatment is unnecessary.

An invasive procedure, such as a splenectomy, is not warranted, because virtually all patients spontaneously recover from chronic neutropenia.

Go to Neutropenia for complete information on this topic.

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Consultations

Hematologic consultation is recommended to exclude other causes of neutropenia and to obtain assistance in the interpretation of neutrophil antibody studies.

Once the diagnosis of autoimmune neutropenia is firmly established, primary care physicians can monitor patients.

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Contributor Information and Disclosures
Author

Susumu Inoue, MD  Professor of Pediatrics and Human Development, Michigan State University College of Human Medicine; Clinical Professor of Pediatrics, Wayne State University School of Medicine; Director of Pediatric Hematology/Oncology, Associate Director of Pediatric Education, Department of Pediatrics, Hurley Medical Center

Susumu Inoue, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Hematology, American Society of Pediatric Hematology/Oncology, International Society for Experimental Hematology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Gary R Jones, MD  Associate Medical Director, Clinical Development, Berlex Laboratories

Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Pharmacy Editor, eMedicine

Disclosure: Nothing to disclose.

Gary D Crouch, MD  Program Director of Pediatric Hematology-Oncology Fellowship, Department of Pediatrics, Associate Professor, Uniformed Services University of the Health Sciences

Gary D Crouch, MD is a member of the following medical societies: American Academy of Pediatrics and American Society of Hematology

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA  Senior Vice President, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University School of Medicine; Clinical Professor of Pediatrics, George Washington University School of Medicine and Health Sciences

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research

Disclosure: Nothing to disclose.

References
  1. Lalezari P, Khorshidi M, Petrosova M. Autoimmune neutropenia of infancy. J Pediatr. Nov 1986;109(5):764-9. [Medline].

  2. Bruin MC, von dem Borne AE, Tamminga RY, Kleijer M, Buddelmeijer L, de Haas M. Neutrophil antibody specificity in different types of childhood autoimmune neutropenia. Blood. Sep 1 1999;94(5):1797-802. [Medline].

  3. Perdikogianni Ch, Dimitriou H, Stiakaki E, Markaki EA, Kalmanti M. Adhesion molecules, endogenous granulocyte colony-stimulating factor levels and replating capacity of progenitors in autoimmune neutropenia of childhood. Acta Paediatr. Nov 2003;92(11):1277-83. [Medline].

  4. Lyall EG, Lucas GF, Eden OB. Autoimmune neutropenia of infancy. J Clin Pathol. May 1992;45(5):431-4. [Medline]. [Full Text].

  5. Sella R, Flomenblit L, Goldstein I, Kaplinsky C. Detection of anti-neutrophil antibodies in autoimmune neutropenia of infancy: a multicenter study. Isr Med Assoc J. Feb 2010;12(2):91-6. [Medline].

  6. Denic S, Showqi S, Klein C, Takala M, Nagelkerke N, Agarwal MM. Prevalence, phenotype and inheritance of benign neutropenia in Arabs. BMC Blood Disord. Mar 27 2009;9:3. [Medline]. [Full Text].

  7. Vlacha V, Feketea G. The clinical significance of non-malignant neutropenia in hospitalized children. Ann Hematol. Dec 2007;86(12):865-70. [Medline].

  8. Hsieh MM, Everhart JE, Byrd-Holt DD, Tisdale JF, Rodgers GP. Prevalence of neutropenia in the U.S. population: age, sex, smoking status, and ethnic differences. Ann Intern Med. Apr 3 2007;146(7):486-92. [Medline].

  9. Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. Jan 1 1998;91(1):181-6. [Medline].

  10. Jonsson OG, Buchanan GR. Chronic neutropenia during childhood. A 13-year experience in a single institution. Am J Dis Child. Feb 1991;145(2):232-5. [Medline].

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A case of secondary autoimmune neutropenia. This patient presented with recurrent otitis and areas of cellulitis in the diaper area. Pseudomonas aeruginosa and Staphylococcus aureus were isolated from the skin lesions. Autoimmune hemolytic anemia and autoimmune neutropenia were confirmed based on the presence of autoantibodies. The patient has a mutation on exon 15, A504T, which changed an asparagine residue to a valine residue.
 
 
 
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