Pediatric Autoimmune and Chronic Benign Neutropenia Medication
- Author: Susumu Inoue, MD; Chief Editor: Max J Coppes, MD, PhD, MBA more...
Medication Summary
As previously stated, in patients with frequent infections, prophylactic antibiotics with trimethoprim and sulfamethoxazole may help.
G-CSF has been demonstrated to raise neutrophil counts and may be useful for the treatment of persistent infections. Intravenous gammaglobulin may be used for the same purpose. Reserve these medications for infections that do not respond to conventional antibiotics.
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.
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.
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).
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.
Lalezari P, Khorshidi M, Petrosova M. Autoimmune neutropenia of infancy. J Pediatr. Nov 1986;109(5):764-9. [Medline].
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].
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].
Lyall EG, Lucas GF, Eden OB. Autoimmune neutropenia of infancy. J Clin Pathol. May 1992;45(5):431-4. [Medline]. [Full Text].
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].
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].
Vlacha V, Feketea G. The clinical significance of non-malignant neutropenia in hospitalized children. Ann Hematol. Dec 2007;86(12):865-70. [Medline].
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].
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].
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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