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 the benefits are anecdotal,^[9]and no randomized, controlled trials have been done. In children with recurrent infections, treatment with G-CSF (Neupogen) greatly decreases frequency of infections.

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.

Spontaneous recovery after 6-24 months is typical. If it persists beyond age 4-5 years, consider other diagnoses. It is important to follow these patients into recovery for this reason.

Go to Neutropenia for complete information on this topic.

Consultations

Hematologic consultation is recommended to exclude other causes of neutropenia, in particular severe congenital neutropenia and acute lymphoblastic leukemia, 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.

Medication

Dale DC, Bolyard AA. An update on the diagnosis and treatment of chronic idiopathic neutropenia. Curr Opin Hematol. 2017 Jan. 24 (1):46-53. [QxMD MEDLINE Link].
Wang LY, Wang CL, Chu CC, et al. Primary autoimmune neutropenia in children in Taiwan. Transfusion. 2009 May. 49 (5):1003-6. [QxMD MEDLINE Link].
Farruggia P, Puccio G, Fioredda F, et al. Autoimmune neutropenia of childhood secondary to other autoimmune disorders: Data from the Italian neutropenia registry. Am J Hematol. 2017 Sep. 92 (9):E546-9. [QxMD MEDLINE Link]. [Full Text].
Porretti L, Farruggia P, Colombo FS, et al. Diagnostic value of cell bound and circulating neutrophil antibody detection in pediatric neutropenia. Pediatr Blood Cancer. 2018 Apr. 65 (4):[QxMD MEDLINE Link].
Ito T, Taniuchi S, Tsuji S, Iharada A, Hasui M, Kaneko K. Diagnosis of autoimmune neutropenia by neutrophil-bound IgG and IgM antibodies. J Pediatr Hematol Oncol. 2011 Oct. 33 (7):552-5. [QxMD MEDLINE Link].
Onodera R, Kurita E, Taniguchi K, et al. Anti-human neutrophil antigen-1a, -1b, and -2 antibodies in neonates and children with immune neutropenias analyzed by extracted granulocyte antigen immunofluorescence assay. Transfusion. 2017 Nov. 57 (11):2586-94. [QxMD MEDLINE Link].
Boxer LA, Bolyard AA, Marrero TM, et al. Is there a role for anti-neutrophil antibody testing in predicting spontaneous resolution of neutropenia in young children. Blood. 2015. 126 (23):2211.
Angelino G, Caruso R, D'Argenio P, et al. Etiology, clinical outcome, and laboratory features in children with neutropenia: analysis of 104 cases. Pediatr Allergy Immunol. 2014 May. 25 (3):283-9. [QxMD MEDLINE Link].
Bruin M, Dassen A, Pajkrt D, Buddelmeyer L, Kuijpers T, de Haas M. Primary autoimmune neutropenia in children: a study of neutrophil antibodies and clinical course. Vox Sang. 2005 Jan. 88(1):52-9. [QxMD MEDLINE Link].
Adamiak T, Gheller-Rigoni A, Arca M, Grossman W, Goday PS. Perianal disease as the initial presentation of autoimmune neutropenia. J Pediatr Gastroenterol Nutr. 2010 Jan. 50 (1):99-102. [QxMD MEDLINE Link]. [Full Text].
Tokushima-Imayoshi M, Onoue T, Matsuo M, et al. Autoimmune neutropenia of infancy with multiple brain abscesses during the course of human herpesvirus-6 infection. Int J Hematol. 2006 Aug. 84 (2):151-3. [QxMD MEDLINE Link].
Al Ghaithi I, Wright NA, Breakey VR, et al. Combined Autoimmune Cytopenias Presenting in Childhood. Pediatr Blood Cancer. 2016 Feb. 63 (2):292-8. [QxMD MEDLINE Link].
Farmer JR, Foldvari Z, Ujhazi B, et al. Outcomes and Treatment Strategies for Autoimmunity and Hyperinflammation in Patients with RAG Deficiency. J Allergy Clin Immunol Pract. 2019 Jul - Aug. 7 (6):1970-85.e4. [QxMD MEDLINE Link]. [Full Text].
Lindqvist H, Carlsson G, Moell J, Winiarski J, Sundin M. Neutropenia in childhood: a 5-year experience at a tertiary center. Eur J Pediatr. 2015 Jun. 174 (6):801-7. [QxMD MEDLINE Link].
Lee WI, Chen SH, Huang JL, et al. Identifying patients with neutrophil elastase (ELANE) mutations from patients with a presumptive diagnosis of autoimmune neutropenia. Immunobiology. 2013 May. 218 (5):828-33. [QxMD MEDLINE Link].
Lalezari P, Khorshidi M, Petrosova M. Autoimmune neutropenia of infancy. J Pediatr. 1986 Nov. 109(5):764-9. [QxMD MEDLINE Link].
Nakamura K, Miki M, Mizoguchi Y, Karakawa S, Sato T, Kobayashi M. Deficiency of regulatory T cells in children with autoimmune neutropenia. Br J Haematol. 2009 Jun. 145(5):642-7. [QxMD MEDLINE Link].
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. 1999 Sep 1. 94(5):1797-802. [QxMD MEDLINE Link].
Urban C, Lackner H, Sovinz P, Beham-Schmid C. Intramedullary aggregation and phagocytosis of neutrophils in chronic benign neutropenia of childhood. Br J Haematol. 2008 Aug. 142(4):504. [QxMD MEDLINE Link].
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. 2003 Nov. 92(11):1277-83. [QxMD MEDLINE Link].
Kobayashi M, Ueda K, Kojima S, Nishihira H, Ishiguro A, Shimbo T. Serum granulocyte colony-stimulating factor levels in patients with chronic neutropenia of childhood: modulation of G-CSF levels by myeloid precursor cell mass. Br J Haematol. 1999 May. 105(2):486-90. [QxMD MEDLINE Link].
Kløve-Mogensen K, Steffensen R, Masmas TN, et al. ABO, secretor, and Lewis carbohydrate histo-blood groups are associated with autoimmune neutropenia of early childhood in Danish patients. Transfusion. 2022 Aug. 62 (8):1636-42. [QxMD MEDLINE Link]. [Full Text].
Lyall EG, Lucas GF, Eden OB. Autoimmune neutropenia of infancy. J Clin Pathol. 1992 May. 45(5):431-4. [QxMD MEDLINE Link]. [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. 2010 Feb. 12(2):91-6. [QxMD MEDLINE Link].
Denic S, Showqi S, Klein C, Takala M, Nagelkerke N, Agarwal MM. Prevalence, phenotype and inheritance of benign neutropenia in Arabs. BMC Blood Disord. 2009 Mar 27. 9:3. [QxMD MEDLINE Link]. [Full Text].
Ortiz MV, Meier ER, Hsieh MM. Identification and Clinical Characterization of Children With Benign Ethnic Neutropenia. J Pediatr Hematol Oncol. 2016 Apr. 38 (3):e140-3. [QxMD MEDLINE Link]. [Full Text].
Grann VR, Ziv E, Joseph CK, Neugut AI, Wei Y, Jacobson JS. Duffy (Fy), DARC, and neutropenia among women from the United States, Europe and the Caribbean. Br J Haematol. 2008 Oct. 143(2):288-93. [QxMD MEDLINE Link].
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. 2010 Feb. 12(2):91-6. [QxMD MEDLINE Link].
Hajishengallis E, Rashewsky S, Kulkarni C, Stathopoulou P. Autoimmune Neutropenia as a Cause of Periodontal Disease in Preschool Children. J Clin Pediatr Dent. 2016 Winter. 40 (1):69-75. [QxMD MEDLINE Link].
Shete M, Thompson JW, Naidu SI, Stocks RM, Wang WC. Olaryngologic manifestations in children with chronic neutropenia. Int J Pediatr Otorhinolaryngol. 2012 Mar. 76(3):392-5. [QxMD MEDLINE Link].
Fioredda F, Calvillo M, Burlando O, et al. Infectious complications in children with severe congenital, autoimmune or idiopathic neutropenia: a retrospective study from the Italian Neutropenia Registry. Pediatr Infect Dis J. 2013 Apr. 32 (4):410-2. [QxMD MEDLINE Link].
Bejjani N, Beaupain B, Bertrand Y, Bellanne-Chantelot C, Donadieu J. How to differentiate congenital from noncongenital chronic neutropenia at the first medical examination? Proposal of score: A pilot study from the French Severe Chronic Neutropenia registry. Pediatr Blood Cancer. 2017 Dec. 64 (12):[QxMD MEDLINE Link].
Vlacha V, Feketea G. The clinical significance of non-malignant neutropenia in hospitalized children. Ann Hematol. 2007 Dec. 86(12):865-70. [QxMD MEDLINE Link].
Alexandropoulou O, Kossiva L, Haliotis F, et al. Transient neutropenia in children with febrile illness and associated infectious agents: 2 years' follow-up. Eur J Pediatr. 2013 Jun. 172 (6):811-9. [QxMD MEDLINE Link].
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. 2007 Apr 3. 146(7):486-92. [QxMD MEDLINE Link].
Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. 1998 Jan 1. 91(1):181-6. [QxMD MEDLINE Link].
Audrain M, Martin J, Fromont P, Prie N, Thomas C, Muller EJ. Autoimmune neutropenia in children: analysis of 116 cases. Pediatr Allergy Immunol. 2011 Aug. 22(5):494-6. [QxMD MEDLINE Link].
Farruggia P, Dufour C. Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians. Ther Adv Hematol. 2015 Feb. 6 (1):15-24. [QxMD MEDLINE Link].
Lane SW, Hassell P, Kennedy GA, Fung YL, Williams BA. Characterization of the bone marrow immunofluorescence test in childhood autoimmune neutropenia. Int J Lab Hematol. 2009 Oct. 31 (5):567-71. [QxMD MEDLINE Link].
Nixon CP, Sweeney JD. Autoimmune Cytopenias: Diagnosis & Management. R I Med J (2013). 2016 Dec 1. 99 (12):36-40. [QxMD MEDLINE Link]. [Full Text].

Media Gallery

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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