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Autoimmune Neutropenia Associated With Callejas Caballero et al. BMC Infect Dis (2021) 21:830 https://doi.org/10.1186/s12879-021-06506-9 CASE REPORT Open Access Autoimmune neutropenia associated with infuenza virus infection in childhood: a case report Ignacio Callejas Caballero1* , Marta Illán Ramos1, Arantxa Berzosa Sánchez1, Eduardo Anguita2,3 and José Tomás Ramos Amador1 Abstract Background: Although neutropenia is relatively frequent in infants and children and is mostly a benign condition with a self-limited course, it can lead to life-threatening severe infections. Autoimmune neutropenia is a relatively uncommon hematological disorder characterized by the autoantibody- induced destruction of neutrophils. It is usually triggered by viral infections with very few documented cases after infuenza virus. Case presentation: An 8-month-old male infant presented at the emergency room with a 5-days history of fever up to 39.7 °C, cough and runny nose. In the blood test performed, severe neutropenia was diagnosed (neutrophils 109/μL). A nasopharyngeal aspirate revealed a positive rapid test for Infuenza A. Serum antineutrophil antibodies were determined with positive results. Neutropenia targeted panel showed no mutations. Despite maintenance of severe neutropenia for 9 months the course was uneventful without treatment. Conclusions: When severe neutropenia is diagnosed and confrmed, it is essential to rule out some potential etiolo- gies and underlying conditions, since the appropriate subsequent management will depend on it. Although autoimmune neutropenia triggered by viral infections has been widely reported, it has seldom been reported after infuenza infection. The benign course of the disease allows a conservative management in most cases. Keywords: Autoimmune neutropenia, Childhood, Infuenza infection, Case report Background Te cause of neutropenia can be primary or sec- Te diagnosis and management of neutropenia in chil- ondary to a broad spectrum of underlying diseases, dren is a real challenge for pediatricians. Te diferential including immunodefciency, rheumathological dis- diagnosis is complex and there is a potential risk of seri- ease, lymphoproliferative disorders, solid tumors or ous complications, including mortality in some cases. drug-induced. In the pediatric age, primary autoimmune cytopenia with a single line cell involvement are much more fre- *Correspondence: [email protected] quent than those with a secondary origin. Autoimmune 1 Department of Paediatrics, Hospital Universitario Clínico San Carlos, thrombocytopenia and hemolytic anemia are much Madrid, Spain Full list of author information is available at the end of the article more common than autoimmune neutropenia. Te © The Author(s) 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http:// creat iveco mmons. org/ licen ses/ by/4. 0/. The Creative Commons Public Domain Dedication waiver (http:// creat iveco mmons. org/ publi cdoma in/ zero/1. 0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data. Callejas Caballero et al. BMC Infect Dis (2021) 21:830 Page 2 of 5 involvement of more than one cell line is usually due to a Leukocytes 7800/μL (neutrophils 109/μL, 1.4%; lym- predisposing secondary cause warranting a more exten- phocytes 6630/μL, 85.2%; monocytes 1030/μL, 13.2%; sive diagnosis work-up. eosinophils 7.8/μL, 0.1%; basophils 7.8/μL, 0.1%). Te Autoimmune neutropenia is usually a benign process biochemical tests revealed normal glucose, creatinine starting more frequently in toddlers, in which the auto- and aminotranferases. Both C-reactive protein (CRP) immune nature is infrequently determined. Furthermore, and procalcitonin (PCT) were within normal limits the triggering etiology is seldom recognized. Tere is lit- (CRP < 0.29 mg/dL, PCT 0.06 ng/mL). tle information regarding the autoimmune cause due to A nasopharyngeal aspirate obtained at the emergency the difculty in diagnosis and very scarce data on the room revealed a positive rapid test for Infuenza A and triggering etiology. negative for RSV. Te blood culture was drawn and It is usually triggered by viral infections [1–4] with very turned out to be negative. few documented cases after infuenza virus [5]. We pre- After diagnosing the severe neutropenia, the mother sent here the case of an infant diagnosed of autoimmune told us the infant had adequate height and weight gain, neutropenia after infection by the Infuenza A virus. without relevant infections or alterations in stools. No drugs in the last days-weeks were given, but paraceta- mol in the current episode. He did not present dysmor- Case presentation phic features. No family history of autoimmunity was An 8-month-old male infant presented at the emergency reported. Te patient was discharged from the emer- room in March 2020 with a 5-days history of fever up to gency room with a diagnosis of neutropenia and infu- 39.7 °C, cough, runny nose and occasional vomiting. enza A infection and referred to the outpatient clinic for Te mother reported mild asthenia and partial decrease further evaluation. in oral intake. Once neutropenia was confrmed 1 month after diag- He did not attend daycare. He did not receive any drug nosis, hemograms were performed every 7–10 days in but paracetamol in the current episode to control the the following month, in which the number of neutrophils fever. Te patient was still breast-fed and the mother was always between 100 and 400/μL. Tere was no ane- reported mild cough and runny nose with no fever (or mia or thrombocytopenia. Immunoglobulins levels, vita- fu-like symptoms). min B12, and folic acid were within the normal range for Personal and family history was unremarkable. Tere age. was a controlled pregnancy, delivering by caesarian sec- SARS-CoV2 serology 1 month after going to the emer- tion at 39 weeks of gestational age. No resuscitation was gency room resulted negative. In addition, serologies to required and no infectious risk factors were identifed. CMV, EBV, parvovirus B19, HIV, Mycoplasma pneumo- Te neonatal period was uneventful with normal niae and Chlamydia pneumoniae were also negative. weight gain and development. Vaccinations were up to Granulocyte-specifc antibodies were detected by fow date according to the Community of Madrid calendar, cytometric assay and Luminex-based multiplex fuores- including also rotavirus and 4CMenB without remark- cent bead platform (LABScreen™ Multi, One Lambda able side-efects. Te patient was exclusively breast-fed Termo Fisher Scientifc) at 10 and 16 months of age during the frst 6 months of life, with adequate weight for detection against HLA-Class I, Class II and HNA gain, and normal stool consistency. Tere was no history Antigens. of mouth ulcers or relevant infectious episodes. Te child remained asymptomatic at all hospital visits. No previous blood tests were available, since the During the 14 months of follow up since diagnosis the patient had never before undergone blood drawn. patient has had an uneventful course with normal growth On physical exam at the emergency room, the tem- and development. He has completed vaccinations includ- perature was 36.9 °C, oxygen saturation 98% with room ing MMR, chickenpox and infuenzae without complica- air and heart rate 155 bpm. Te child was in good gen- tions. At the age of 14 months he started daycare having eral condition, well hydrated and perfused with no signs two mild upper respiratory episodes, one of them associ- of respiratory distress. Te mouth was normal without ated with COVID-19 confrmed by nasopharyngeal aspi- sores, ulcers or exudates. Te lung auscultation revealed rate PCR and positive serology. mild hypoventilation in the left base. A thorough genetic NGS panel for congenital neutro- Te rest of the physical exam was considered normal. penia, including the following genes was performed by In the emergency department, a chest radiograph DNA target sequence enrichment capture with Miseq was performed, showing difuse retrocardiac consoli- illumine analysis, being all unmutated: AK2, AP3B1, dation. Blood tests were requested with the following BLOC1S3, CDAN1, CLPB, COH1/VPS13B, CSF3R, parameters: CXCR4, DTNBP1/BLOC1S8, DYN2/DNM2, EIF2AK3/ Callejas Caballero et al. BMC Infect Dis (2021) 21:830 Page 3 of 5 PERK, ELANE/ELA2, G6PT1/SLC37A4, G6PC3, GATA- respectively [6, 7]. Although sometimes a genetic panel 1, GFI1, HAX1, HPS1, HPS3, HPS4, HPS5, HPS6, JAGN1, to detect the mutation of common genes associated with LYST, MAPBPIP/LAMTOR2, MYO5A, PLDN/BLOC1S6, congenital neutropenia is performed [8], it is not usually RAB27A, RMRP/CHH/NME1, SEC23B, SLC19A2, necessary when there is a high suspicion of autoimmune SLC25A38, SLC37A4, SMARCAL1, TAZ, TCIRG1, USB1, neutropenia [7] VPS13B, VPS45, WAS/SCNX. Children with autoimmune neutropenia rarely suf- At the age of 21 months, a blood count showed that the fer from serious infections, since circulating neutro- patient had recovered the neutrophil count (total ANC phils, although low in number, are normally functional, 1100/μL). and therefore capable of defending the host against such infections. Furthermore, the severity and type of infec- Discussion and conclusions tions are poorly related with the neutrophil count. It is Infuenza A as a trigger for neutropenia has been important to investigate other fndings that may rise the reported so far in just a few cases [5].
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