Neutropenia in children: acquired neutropenia
DOI:
https://doi.org/10.15574/SP.2021.117.66Keywords:
neutropenia, children, immunodeficiency, autoimmune neutropeniaAbstract
Neutropenia is defined as a reduction in the absolute neutrophil count (ANC) below 1,500 cells/mcl in the blood. Neutropenia is a common laboratory finding in children. It is important to distinguish transient and benign causes from severe congenital neutropenia. Neutropenia can be classified in asymptomatic (mild), moderate, and severe form, the susceptibility to infection depending on ANC. Neutropenia can be either acquired or congenital. Infection, drugs, and immune disorders are the most common acquired causes while congenital causes are rare and confined mostly to infants and children.
Transient neutropenia often accompanies viral infections in children, manifested in the period of acute viremia. Young children are characterized by autoimmune neutropenia (AIN), which has a benign course and a favorable prognosis. Autoimmune neutropenia is characterized by a decrease in the number of neutrophils as a result of the destruction of neutrophils in the peripheral blood by antineutrophil antibodies. The duration of AIN is usually 3–5 years. This is a self-limiting disease that in most cases does not require treatment. Despite the benign course, serious infectious complications can occur.
Treatment of myeloid growth factors should be started after a previous bone marrow aspiration biopsy in children with severe infections or requiring surgical intervention. High doses of intravenous immunoglobulin and corticosteroids may be effective in treating AIN in patients with life-threating infections. The danger to the patient depends on the etiology, ANC and bone marrow status. The risk of infections is significantly increased in patients with ACN less than 500 cells/μ1. The most common loci of infections include the oral mucosa, skin, perirectal area, perineum. Oral ulcers and gingivitis are characteristic signs of clinically significant neutropenia, requiring the exclusion of its congenital causes. Severe infections in patients with neutropenia are caused by pyogenic or intestinal bacteria and Candida species.
It is important to distinguish between transient or benign causes and severe congenital neutropenia or neutropenia associated with serious haematological or systemic disease. Clarification of the cause of neutropenia is important for determining management and prognosis.
No conflict of interest was declared by the author.
References
Audrain M, Martin J, Fromont P et al. (2011). Autoimmune neutropenia in children: Analysis of 116 cases. Pediatr Allergy Immunol. 22: 494-496. https://doi.org/10.1111/j.1399-3038.2010.01117.x; PMid:21771084
Autrel-Moignet A, Lamy T. (2014). Autoimmune neutropenia. Presse Med. 43: e105-118. https://doi.org/10.1016/j.lpm.2014.02.007; PMid:24680423
Borregaard N. (2010). Neutrophils, from Marrow to Microbes. Immunity. 33: 657-670. https://doi.org/10.1016/j.immuni.2010.11.011; PMid:21094463
Boxer LA. (2012). How to approach neutropenia Hematology: 174-182. https://doi.org/10.1182/asheducation.V2012.1.174.3798251; PMid:23233578
Bux J, Behrens G, Jaeger G, Welte K. (1998). Diagnosis and clinical course of autoimmune neutropenia in infancy: analysis of 240 cases. Blood. 91: 181-186. https://doi.org/10.1182/blood.V91.1.181; PMid:9414283
Celkan T, Koc BS. (2015). Approach to the patient with neutropenia in childhood. Turk Pediatri Ars. 50: 136-144. https://doi.org/10.5152/TurkPediatriArs.2015.2295; PMid:26568688 PMCid:PMC4629920
Chernyshova LI, Volokha AP, Kostiuchenko LV. (2013). Dytiacha imunolohiia: pidruchnyk. Za red. prof. L.I. Chernyshovoi, A.P. Volokhy. K.: VSV «Medytsyna»: 720.
Dale DC, Bolyard AA. (2017). An update on the diagnosis and treatment of chronic idiopathic neutropenia. Curr Opin Hematol. 24: 46-53. https://doi.org/10.1097/MOH.0000000000000305; PMid:27841775 PMCid:PMC5380401
Dale DC. (2016). How I diagnose and treat neutropenia. Curr Opin Hematol. 23: 1-4. https://doi.org/10.1097/MOH.0000000000000208; PMid:26554885 PMCid:PMC4668211
Farruggia P, Dufour C. (2015). Diagnosis and management of primary autoimmune neutropenia in children: insights for clinicians. Ther Adv Hematol. 6 (1): 15-24. https://doi.org/10.1177/2040620714556642; PMid:25642312 PMCid:PMC4298488
Farruggia P, Fioredda F, Puccio G et al. (2015). Autoimmune neutropenia of infancy: Data from the Italian neutropenia registry. Am J Hematol. 90: E221-E222. https://doi.org/10.1002/ajh.24187; PMid:26361081
Farruggia P. (2016). Immune neutropenias of infancy and childhood. World J Pediatr. 12: 142. https://doi.org/10.1007/s12519-015-0056-9; PMid:26582295
Fioredda F, Calvillo M, Bonanomi S et al. (2011). 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 (Associazione Italiana Emato-Oncologia Pediatrica). Am J Hematol. 57: 10-17. https://doi.org/10.1002/pbc.23108; PMid:21448998
Karakilic-Ozturan E, Karaman S, Soguksu P et al. (2020). The Role of Anti-neutrophil antibodies in the etiologic classification of childhood neutropenia: a cross-sectional study in a tertiary center. J Pediatr Hematol Oncol. 42: 107. https://doi.org/10.1097/MPH.0000000000001710; PMid:31895216
Kyono W, Coates TD. (2002). A practical approach to neutrophil disorders. Pediatr Clin North Am. 49: 929. https://doi.org/10.1016/S0031-3955(02)00030-5
Lehrnbecher T, Robinson P, Fisher B et al. (2017). Guideline for the management of fever and neutropenia in children with cancer and hematopoietic stem-cell transplantation recipients: 2017 Update. J Clin Oncol. 35: 2082-2094. https://doi.org/10.1200/JCO.2016.71.7017; PMid:28459614
Liles WC, Starkebaum G, Dale DC. (2004). Neutropenia in systemic lupus erythematosus. Blood. 104: 2. https://doi.org/10.1182/blood-2004-04-1546
MOZ Ukrainy. (2020). Diahnostyka neitropenii u ditei. Klinichna nastanova, zasnovana na dokazakh. Modern Pediatrics. Ukraine. 1 (105): 89-103.
Newburger PE, Dale DC. (2013, Jul). Evaluation and management of patients with isolated neutropenia. Semin Hematol. 50: 198-206. https://doi.org/10.1053/j.seminhematol.2013.06.010; PMid:23953336 PMCid:PMC3748385
Newburger PE. (2016). Autoimmune and other acquired neutropenias. Hematology Am Soc Hematol Educ Program. 2016: 38-42. https://doi.org/10.1182/asheducation-2016.1.38; PMid:27913460 PMCid:PMC5380382
Segel GB, Halterman JS. (2008). Neutropenia in pediatric practice. Pediatrics in Review. 29 (1): 12-24. https://doi.org/10.1542/pir.29-1-12; PMid:18166617
Sella R, Flomenblit L, Goldstein I, Kaplinsky C. (2010). Detection of antineutrophil antibodies in autoimmune neutropenia of infancy: a multicenter study. Isr Med Assoc J. 12: 91.
Sicre de Fontbrune F, Moignet A, Beaupain B et al. (2015). Severe chronic primary neutropenia in adults: report on a series of 108 patients. Blood. 126: 1643. https://doi.org/10.1182/blood-2015-03-634493; PMid:26261239
Starkebaum G. (2002). Chronic neutropenia associated with autoimmune disease. Semin Hematol. 39: 121. https://doi.org/10.1053/shem.2002.31918; PMid:11957195
Szymon Janczar1, Beata Zalewska-Szewczyk, Katarzyna Babol-Pokora. (2020). Vaccination in children with chronic severe neutropenia - review of recommendations and a practical approach. Cent Eur J Immunol. 45 (2): 202-205. https://doi.org/10.5114/ceji.2020.97910; PMid:33456332 PMCid:PMC7792435
Teachey DT, Lambert MP. (2013). Diagnosis and Management of Autoimmune Cytopenias in Childhood. Pediatr Clin N Am. 60: 1489-1511. https://doi.org/10.1016/j.pcl.2013.08.009; PMid:24237984 PMCid:PMC5384653
Thomas AE, Simpson LA. (2017). A step-by-step approach to paediatric neutropenia. Pediatr Child Health. 27: 511-516. https://doi.org/10.1016/j.paed.2017.07.006
Volokha AP, Bondarenko AV, Boiarchuk OR. (2020). Vaktsynatsiia ditei z pervynnymy imunodefitsytamy. Metodychni rekomendatsii: 25.
Walkovich K, Boxer LA. (2013). How to approach neutropenia in childhood. Pediatrics in Review. 34 (4): 173-184. https://doi.org/10.1542/pir.34-4-173; PMid:23547064
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