Pediatric Autoimmune and Chronic Benign Neutropenia Clinical Presentation

Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...

Updated: Mar 29, 2011

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History
Physical Examination
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History

Most children with autoimmune neutropenia receive initial medical attention because of the occurrence of a febrile illness during the last 6 months of infancy. Such illnesses include the following:

A simple febrile illness
Acute otitis media
Upper respiratory tract infection
Pneumonia
Gastroenteritis
Tonsillitis/pharyngitis
Skin infection

No family history of neutropenia or leukopenia is reported, and if a complete blood count (CBC) is performed earlier in the child's life, it is usually within the reference range.

Children with autoimmune neutropenia may have a history of frequent upper respiratory infections. Some patients are asymptomatic. A CBC count performed for an unrelated reason may reveal neutropenia.

Physical Examination

Physical examination may reveal signs of a local infection, including mouth ulcer; gingivitis; upper respiratory infections; impetigo; otitis media; and, rarely, cellulitis, abscesses, or sepsis.

Many children may simply present with fever without any focal infection.

Patients generally do not exhibit growth failure or chronic illness.

Proceed to Differential Diagnoses

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

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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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