Case Series

Identification of Antibodies against Neutrophil Surface Antigens in Two Iranian Patients with Autoimmune Neutropenia

Anti-neutrophil Antibodies in Autoimmune Neutropenia


Autoimmune neutropenia is a type of immune-mediated neutropenia, caused by antibody-induced neutrophil destruction. Here we report two cases (3-year-old boy and 9-year-old girl) with suspected autoimmune neutropenia. The presence of neutrophil antibodies in sera of these patients was investigated using standard neutrophil antibody screening tests such as granulocyte immunofluorescence test (GIFT), granulocyte agglutination test (GAT), and lymphocyte immunofluorescence test (LIFT). A positive reactivity with two panel cells was found in GIFT. No reactivities with panel cells were observed in GAT and LIFT. To the best of our knowledge, this is the first report for detecting the neutrophil reactive antibodies using genotyped neutrophils in patients with autoimmune neutropenia in Iran. The final diagnosis of our patients was primary autoimmune neutropenia for the boy and autoimmune neutropenia associated with familial Mediterranean fever for the girl.

1. Rosales C. Neutrophil: a cell with many roles in inflammation or several cell types? Front physiol. 2018;9(5):113-9.
2. Frater JL. How I investigate neutropenia. Int J Lab Hematol. 2020;42(4):121-32.
3. Autrel-Moignet A, Lamy T. Autoimmune neutropenia. Presse Méd. 2014;43(4):e105-e18.
4. Dale DC. How I diagnose and treat neutropenia. Curr opin hematol. 2016;23(1):1.
5. Matsuhashi M, Tsuno N, Iino J, Nagura Y, Okazaki H, Santoso S. Advances in granulocyte test methodologies. ISBT Sci Ser. 2014;9(1):246-52.
6. Flesch B. Work‐up in the case of granulocyte antibodies. ISBT Sci Ser. 2020;15(1):59-69.
7. Celkan T, Koç BŞ. Approach to the patient with neutropenia in childhood. Turk Pediatri Arş. 2015;50(3):136-41.
8. Flesch BK, Curtis BR, de Haas M, Lucas G, Sachs UJ. Update on the nomenclature of human neutrophil antigens and alleles. Transfusion. 2016;56(6):1477-9.
9. Bux J. Human neutrophil alloantigens. Vox sang. 2008;94(4):277-85.
10. Moritz E, Norcia ÂM, Cardone JD, Kuwano ST, Chiba AK, Yamamoto M, et al. Human neutrophil alloantigens systems. An Acad Bras Ciênc. 2009;81(3):559-69.
11. Afzal W, Owlia MB, Hasni S, Newman KA. Autoimmune Neutropenia Updates: Etiology, Pathology, and Treatment. South med J. 2017;110(4):300-7.
12. Capsoni F, Sarzi-Puttini P, Zanella A. Primary and secondary autoimmune neutropenia. Arthritis res ther. 2005;7(5):208-11.
13. Bux J, Behrens G, Jaeger G, Welte K. Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. 1998;91(1):181-6.
14. Lyall E, Lucas G, Eden O. Autoimmune neutropenia of infancy. J clin pathol. 1992;45(5):431-4.
15. 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. 2013;32(4):410-2.
16. Holland SM, Uzel G. Phagocyte deficiencies. Clin Immunol. 2019;319-33.
17. Chaudhari PM, Budh DP. Autoimmune And Chronic Neutropenia. StatPearls [Internet]. 2020.
18. Padeh S, Bilginer Y, Ozen S. Familial mediterranean fever. Textbook of Autoinflammation: Springer; 2019. p. 293-313.
19. Church LD, Churchman SM, Hawkins PN, McDermott MF, editors. Hereditary auto-inflammatory disorders and biologics. Springer semin immunopathol; 2006: Springer.
20. Manukyan G, Aminov R, Hakobyan G, Davtyan T. Accelerated apoptosis of neutrophils in familial Mediterranean fever. Front immunol. 2015;6(2):239-41.
21. Lucas G, Porcelijn L, Fung YL, Green F, Reil A, Hopkins M, et al. External quality assessment of human neutrophil antigen (HNA)‐specific antibody detection and HNA genotyping from 2000 to 2012. Vox sang. 2013;105(3):259-69.
22. Denic S, Narchi H, Al Mekaini LA, Al-Hammadi S, Al Jabri ON, Souid A-K. Prevalence of neutropenia in children by nationality. BMC hematol. 2016;16(1):15-8.
23. Salehi T, Fazlollahi MR, Maddah M, Nayebpour M, Yazdi MT, Alizadeh Z, et al. Prevention and control of infections in patients with severe congenital neutropenia; a follow up study. Iran J Allergy Asthma Immunol. 2012;11(1):51-5.
IssueVol 20 No 6 (2021) QRcode
SectionCase Series
Agglutination test Autoimmunity Indirect immunofluorescence Neutropenia

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How to Cite
Esmaeili B, Bayat B, Fazlollahi MR, Moradi L, Kalantari A, Nourbakhsh SMK, Pourpak Z. Identification of Antibodies against Neutrophil Surface Antigens in Two Iranian Patients with Autoimmune Neutropenia. Iran J Allergy Asthma Immunol. 2021;20(6):771-777.