Evaluation and Management of Autoimmune Neutropenia

  • Alicia K. ChangEmail author


An evaluation into the underlying cause of neutropenia is important, as this may predict clinical severity and guide anticipatory guidance for families. A thorough medical history and physical examination can often narrow the differential in a child with neutropenia, and laboratory testing is typically reserved for those with a more severe or atypical presentation. Neutrophil autoantibodies remain difficult to detect with current methods and require a combination of agglutination and immunofluorescence testing.


Neutropenia Human neutrophil alloantigens 


  1. 1.
    Dale DC. How I manage children with neutropenia. Br J Haematol. 2017;178(3):351–63.CrossRefPubMedGoogle Scholar
  2. 2.
    Afzal W, Owlia MB, Hasni S. Autoimmune neutropenia updates: etiology, pathology and treatment. South Med J. 2017;110(4):300–7.CrossRefPubMedGoogle Scholar
  3. 3.
    Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. 1998;91:181–6.PubMedPubMedCentralGoogle Scholar
  4. 4.
    Capsoni F, Sarzi-Puttini P, Zanella A. Primary and secondary autoimmune neutropenia. Arthritis Res Ther. 2005;7(50):208–14.CrossRefPubMedPubMedCentralGoogle Scholar
  5. 5.
    Newburger PE. Autoimmune and other acquired neutropenias. Hematol Am Soc Hematol Educ Prog. 2016;2016(1):38–42.Google Scholar
  6. 6.
    Nossent JC, Swaak AJ. Prevalence and significance of haematological abnormalities in patients with systemic lupus erythematosus. Q J Med. 1991;80:605–12.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Starkebaum G, Arend WP. Neutrophil-binding immunoglobulin G in systemic lupus erythematosus. J Clin Invest. 1979;64:902–12.CrossRefPubMedPubMedCentralGoogle Scholar
  8. 8.
    Dale DC, Welte K. Cyclic and chronic neutropenia. Cancer Treat Res. 2011;157:97–108.CrossRefPubMedGoogle Scholar
  9. 9.
    Farrugia P, Dufour C. Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians. Ther Adv Hematol. 2015;6(1):15–24.CrossRefGoogle Scholar
  10. 10.
    Stroncek D. Granulocyte gene polymorphisms and mutations: effects on immune response and neutrophil proliferation. Vox Sang. 2004;87(Suppl 2):101–4.CrossRefPubMedGoogle Scholar
  11. 11.
    Rosenberg PS, Zeidler C, Bolyard AA, Alter BP, Bonilla MA, Boxer LA, et al. Stable long-term risk of leukemia in patients with severe congenital neutropenia maintained on G-CSF therapy. Br J Haematol. 2010;150:196–9.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Andres E, Zimmer J, Affenberger S. Idiosyncratic drug-induced agranulocytosis: update of an old disorder. Eur J Intern Med. 2006;17:529–35.CrossRefPubMedGoogle Scholar
  13. 13.
    Bruin MCA, Borne AE, Tamminga RY, Kleijer M, Buddelmeijer L, de Haas M. Neutrophil antibody specificity in different types of childhood autoimmune neutropenia. Blood. 1999;94(5):1797–802.PubMedPubMedCentralGoogle Scholar
  14. 14.
    Boxer LA, Greenberg MS, Boxer GJ, Stossel TP. Autoimmune neutropenia. N Engl J Med. 1975;293:748–53.CrossRefPubMedGoogle Scholar
  15. 15.
    Hadley AG, Holburn AM, Bunch C, Chapel H. Anti-granulocyte opsonic activity and autoimmune neutropenia. Br J Haematol. 1986;63(3):581–9.CrossRefPubMedGoogle Scholar
  16. 16.
    Simpson DM, Ross R. Effects of heterologous anti-neutrophil serum in guinea pigs. Hematologic and ultrastructural observations. Am J Pathol. 1971;65:79–102.PubMedPubMedCentralGoogle Scholar
  17. 17.
    Bux J. Molecular Nature of Antigens implicated in immune neutropenias. International Journal Hematology. 2002;76 Suppl 1:399–403.CrossRefPubMedGoogle Scholar
  18. 18.
    Bux J, Chapman J. Report on the second international granulocyte serology workshop. Transfusion. 1997;37(9):997–83.CrossRefPubMedGoogle Scholar
  19. 19.
    Farrugia P. Immune neutropenias of infancy and childhood. World J Pediatr. 2016;12(2):142–8.CrossRefGoogle Scholar
  20. 20.
    Heinzl MW, Schonbacker M, Dauber E, Panzer S, Mayr WR, Körmöczi GF. Detection of granulocyte-reactive antibodies: a comparison of different methods. Vox Sang. 2015;108:287–93.CrossRefPubMedGoogle Scholar
  21. 21.
    Moritz E, Norcia A, Cardone J, Kuwano ST, Chiba AK, Yamamoto M, et al. Human neutrophil alloantigens systems. An Acad Bras Cienc. 2009;81(3):559–69.CrossRefPubMedGoogle Scholar
  22. 22.
    Bux J. Human neutrophil alloantigens. Vox Sang. 2008;94:277–85.CrossRefPubMedGoogle Scholar
  23. 23.
    Muschter S, Berthold T, Greinacher A. Developments in the definition and clinical impact of human neutrophil antigens. Curr Opin Hematol. 2011;18:452–60.CrossRefPubMedGoogle Scholar
  24. 24.
    Dungerwalla M, Marsh JC, Tooze JA, Lucas G, Ouwehand W, Pettengell R, et al. Lack of clinical efficacy of rituximab in the treatment of autoimmune neutropenia and pure red cell aplasia: implications for their pathophysiology. Ann Hematol. 2007;86(3):191–7.CrossRefGoogle Scholar
  25. 25.
    Fioredda F, Calvillo M, Bananomi S, Coliva T, Tucci F, Farruggia P, et al. Congenital and acquired neutropenias consensus guidelines on therapy and follow-up in childhood from the neutropenia committee of the marrow failure syndrome group of the AIEOP. Am J Hematol. 2012;87(2):238–43.CrossRefPubMedGoogle Scholar
  26. 26.
    Fioredda F, Calvillo M, Burlando O, Riccardi F, Caviglia I, Tucci F, 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;32(4):410–2.CrossRefPubMedGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Section of Hematology-Oncology, Department of PediatricsBaylor College of Medicine, Texas Children’s HospitalHoustonUSA

Personalised recommendations