{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,23]],"date-time":"2026-06-23T08:48:47Z","timestamp":1782204527495,"version":"3.54.5"},"reference-count":36,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T00:00:00Z","timestamp":1761955200000},"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":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2025,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Patients with chronic granulomatous disease (CGD) can develop hemophagocytic lymphohistiocytosis (HLH), exacerbating mortality risk. Despite its clinical significance, data on HLH in CGD from international cohorts remain limited. This study aims to describe the occurrence of HLH in a cohort of patients with CGD, providing clinical insight into this association and emphasizing the need for early recognition and effective management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The records of 60 patients with CGD were reviewed. Those meeting the diagnostic criteria for HLH based on the HScore were included in the analysis. Both descriptive and inferential statistics were employed to evaluate the data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Eleven patients (18.3%) fulfilled the HLH diagnostic criteria. The median interval between CGD genetic diagnosis and HLH onset was 36\u2009months, with a median age at HLH diagnosis of 67\u2009months. Infectious triggers were identified in eight cases, with\n                      <jats:italic>Salmonella<\/jats:italic>\n                      and\n                      <jats:italic>Aspergillus<\/jats:italic>\n                      species being the most common. One case involved an inflammatory trigger\u2014multisystem inflammatory syndrome in children (MIS\u2010C) following SARS\u2010CoV\u20102 infection. Mortality was high: 72.7% of the patients with HLH died. No significant difference (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009=\u2009.338) was observed between those who died after receiving only immunosuppressive therapy (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20092) and those who received both intravenous immunoglobulin and immunosuppressive therapy (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20096).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>HLH in CGD is associated with a high mortality rate. Notably, MIS\u2010C can present as an inflammatory trigger for HLH in this population. Careful evaluation of HLH parameters is recommended for all patients with CGD admitted with infection or inflammation to facilitate early diagnosis and guide management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.70234","type":"journal-article","created":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T08:19:11Z","timestamp":1761985151000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Hemophagocytic lymphohistiocytosis in 60 Mexican children with chronic granulomatous disease"],"prefix":"10.1111","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0009-0003-9622-3785","authenticated-orcid":false,"given":"Alix Sherlyn","family":"Monroy\u2010Garc\u00eda","sequence":"first","affiliation":[{"name":"Department of Clinical Immunology National Institute of Pediatrics  Mexico City Mexico"},{"name":"Laboratory of Immunodeficiency National Institute of Pediatrics  Mexico City 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