{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:58:42Z","timestamp":1790834322900,"version":"4.1.0"},"reference-count":25,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"abstract":"<jats:title>Summary<\/jats:title>\n                  <jats:p>\n                    Genetic neutropenia comprises heterogeneous phenotypes with variable patterns and severity of infectious complications. However, comparative real\u2010world data on subtype\u2010specific infectious complications remain limited. We analysed patients with genetic neutropenia enrolled in the Canadian Inherited Marrow Failure Registry between 2001 and 2025. Patients were classified into severe congenital neutropenia (SCN), cyclic neutropenia, syndromic neutropenia or suspected genetic neutropenia (SGN). Infectious complications were classified by type and severity. Eighty\u2010nine patients were included, including SCN (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200927), cyclic neutropenia (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200913), syndromic neutropenia (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200925) and SGN (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200924).\n                    <jats:italic>Elastase, neutrophil expressed (ELANE)<\/jats:italic>\n                    variants were most common (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200930) and predominated in SCN and cyclic neutropenia, whereas syndromic neutropenia included\n                    <jats:italic>tafazzin (TAZ), solute carrier family 37 member 4 (SLC37A4), vacuolar protein sorting 13 homolog B (VPS13B), C\u2010X\u2010C motif chemokine receptor 4 (CXCR4), glucose\u20106\u2010phosphatase catalytic subunit 3 (G6PC3), signal recognition particle 54 (SRP54)<\/jats:italic>\n                    and\n                    <jats:italic>HCLS1 associated protein X\u20101 (HAX1)<\/jats:italic>\n                    variants. Overall, 68 patients (76.4%) experienced \u22651 infectious complication and 45 (50.6%) developed severe infections. Febrile neutropenia occurred more frequently in SCN (59.3%) than in other subtypes (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.005), while skin and soft tissue infections were more common in syndromic neutropenia (56.0%) and SCN (44.4%) (\n                    <jats:italic>p<\/jats:italic>\n                    \u2009=\u20090.038). Granulocyte colony\u2010stimulating factor was administered in 60 patients, with an overall response rate of 90.0%. Ten patients underwent haematopoietic stem cell transplantation. Infection patterns differed across genetic neutropenia subtypes, supporting subtype\u2010informed surveillance and management.\n                  <\/jats:p>","DOI":"10.1111\/bjh.70823","type":"journal-article","created":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T05:06:57Z","timestamp":1790831217000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical outcome and infectious complications in genetic and suspected genetic neutropenia: A report from the Canadian Inherited Marrow Failure Registry"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7016-8827","authenticated-orcid":false,"given":"Young Bae","family":"Choi","sequence":"first","affiliation":[{"name":"Genetics and Genome Biology Program Hospital for Sick Children  Toronto Ontario Canada"},{"name":"Department of Pediatrics Ajou University School of Medicine  Suwon South Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-8881-7999","authenticated-orcid":false,"given":"Nawaf R. R.","family":"Alshammari","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, College of Medicine University of Ha'il  Ha'il Saudi Arabia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-7542-2125","authenticated-orcid":false,"given":"Rinu","family":"Mathew","sequence":"additional","affiliation":[{"name":"Genetics and Genome Biology Program Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongbing","family":"Li","sequence":"additional","affiliation":[{"name":"Genetics and Genome Biology Program Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michaela","family":"Cada","sequence":"additional","affiliation":[{"name":"Division of Hematology\/Oncology, Department of Pediatrics The Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sharon","family":"Abish","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Montreal Children's Hospital  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yves D.","family":"Pastore","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology CHU Sainte\u2010Justine  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"MacGregor","family":"Steele","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Alberta Children's Hospital  Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6913-2808","authenticated-orcid":false,"given":"Robert J.","family":"Klaassen","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Children's Hospital of Eastern Ontario  Ottawa Ontario 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Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meera","family":"Rayar","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology British Columbia Children's Hospital  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3447-0743","authenticated-orcid":false,"given":"Laura","family":"Wheaton","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Kingston General Hospital  Kingston Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5168-0243","authenticated-orcid":false,"given":"Vicky","family":"Breakey","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology McMaster Children's Hospital  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa","family":"Goodyear","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Janeway Children's Health Centre  St. John's Newfoundland Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4715-4536","authenticated-orcid":false,"given":"Bruce","family":"Crooks","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology IWK Health Centre  Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5804-3116","authenticated-orcid":false,"given":"Soumitra","family":"Tole","sequence":"additional","affiliation":[{"name":"Pediatric Hematology\/Oncology Children's Hospital, London Health Sciences Centre  London Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5047-3493","authenticated-orcid":false,"given":"Ye Jee","family":"Shim","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, School of Medicine Kyungpook National University  Daegu 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