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Pediatric Autoimmune and Chronic Benign Neutropenia Medication

  • Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA  more...
 
Updated: Oct 09, 2015
 

Medication Summary

Patients with frequent infections may benefit from prophylactic antibiotics with trimethoprim and sulfamethoxazole.

G-CSF (Filgrastim, Neupogen) has been demonstrated to raise neutrophil counts and may be useful for the treatment of persistent or recurrent infections. Intravenous gammaglobulin may be used for the same purpose. Reserve these medications for infections that do not respond to conventional antibiotics.

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Colony-Stimulating Factors

Class Summary

Colony-stimulating factors (CSFs) are used for recurrent or refractory infections that are unresponsive to conventional therapy. They act as a hematopoietic growth factor that stimulates the development of granulocytes. CSFs are used to treat or prevent neutropenia in patients who are receiving myelosuppressive cancer chemotherapy and to reduce the period of neutropenia associated with bone marrow transplantation. These agents are also used to mobilize autologous peripheral blood progenitor cells for bone marrow transplantation and in the management of chronic neutropenia.

Filgrastim (G-CSF, Neupogen)

 

This is a G-CSF that activates and stimulates production, maturation, migration, and cytotoxicity of neutrophils. It is recommended only for patients with a clinically significant history of frequent infections.

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Immunoglobulins

Class Summary

Immunoglobulins are used for infections that are unresponsive to conventional measures. Immunoglobulins are used for passive immunization, thus conferring immediate protection against some infectious diseases.

Immune Globulin, Intravenous (Carimune NF, Gammagard S/D)

 

This agent consists of purified IgG from human plasma; all commercially available products are viral inactivated.

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Antibiotics

Class Summary

These agents are used for the prevention of frequent infections.

Trimethoprim and sulfamethoxazole (Bactrim, Septra)

 

This drug combination inhibits bacterial growth by inhibiting the synthesis of dihydrofolic acid. It may help frequent infections (eg, otitis media); however, the dose for this indication has not been established (no clinical studies have demonstrated the efficacy of this drug).

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Corticosteroids

Class Summary

Various therapies are available that may increase the neutrophil count to normal levels temporarily in children with chronic benign neutropenia, which include corticosteroids. Corticosteroids may be useful in patients not responding to other therapies. Routine use of steroids in children with neutropenia is strongly discouraged. Do not use steroids just to increase the counts.

Prednisone

 

Corticosteroids such as prednisone can be used to suppress the antibody formation and increase the neutrophil count. Use of steroids in this disorder is only anecdotal Routine use of steroids in uncomplicated neutropenia is strongly discouraged. Large doses may be required, potentially leading to adverse effects such as increased risk of infection.

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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 Clinical Oncology, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

Disclosure: Nothing to disclose.

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

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

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

Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

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

Disclosure: Nothing to disclose.

Acknowledgements

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.

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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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