{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,19]],"date-time":"2026-05-19T18:57:19Z","timestamp":1779217039161,"version":"3.51.4"},"reference-count":17,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,4,13]],"date-time":"2026-04-13T00:00:00Z","timestamp":1776038400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Med."],"abstract":"<jats:p>\n                    Hemophagocytic lymphohistiocytosis (HLH) secondary to\n                    <jats:italic>Pneumocystis jirovecii<\/jats:italic>\n                    pneumonia (PJP) is extremely rare in children. We present the case of a 10-year-old girl with a history of idiopathic thrombocytopenic purpura (ITP) on long-term oral prednisone, who was admitted for progressive fever, cough, and dyspnea. Metagenomic next-generation sequencing of blood and bronchoalveolar lavage fluid confirmed PJP. Despite targeted antifungal therapy and respiratory support, she developed persistent high-grade fever, pancytopenia, hyperferritinemia, hypofibrinogenemia, and hemophagocytosis on bone marrow aspirate by day 10, meeting diagnostic criteria for HLH. Genetic testing was declined by the parents. Management included dexamethasone, continuous renal replacement therapy, and plasmapheresis. Unfortunately, her condition deteriorated, and she was discharged upon parental request on day 22, succumbing on the same day. To our knowledge, this is the first reported pediatric case of HLH secondary to PJP in China. This case highlights that in children with PJP\u2014especially those on immunosuppressive therapy\u2014the development of persistent fever and cytopenia should prompt immediate evaluation for secondary HLH to enable timely intervention.\n                  <\/jats:p>","DOI":"10.3389\/fmed.2026.1795567","type":"journal-article","created":{"date-parts":[[2026,4,13]],"date-time":"2026-04-13T14:58:40Z","timestamp":1776092320000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Hemophagocytic lymphohistiocytosis secondary to Pneumocystis jirovecii pneumonia: a rare case report"],"prefix":"10.3389","volume":"13","author":[{"given":"Yanqiang","family":"Du","sequence":"first","affiliation":[{"name":"Department of Pediatric Intensive Care Unit of Xi\u2019an Children\u2019s Hospital, National Children\u2019s Regional Medical Center (Northwest), Children\u2019s Hospital of Xi\u2019an Jiaotong University","place":["Xi\u2019an, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhangyan","family":"Guo","sequence":"additional","affiliation":[{"name":"Department of Pediatric Intensive Care Unit of Xi\u2019an Children\u2019s Hospital, National Children\u2019s Regional Medical Center (Northwest), Children\u2019s Hospital of Xi\u2019an Jiaotong University","place":["Xi\u2019an, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dan","family":"Yao","sequence":"additional","affiliation":[{"name":"Department of Pediatric Intensive Care Unit of Xi\u2019an Children\u2019s Hospital, National Children\u2019s Regional Medical Center (Northwest), Children\u2019s Hospital of Xi\u2019an Jiaotong University","place":["Xi\u2019an, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pediatric Intensive Care Unit of Xi\u2019an Children\u2019s Hospital, National Children\u2019s Regional Medical Center (Northwest), Children\u2019s Hospital of Xi\u2019an Jiaotong University","place":["Xi\u2019an, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,4,13]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"124","DOI":"10.1002\/pbc.21039","article-title":"HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.","volume":"48","author":"Henter","year":"2007","journal-title":"Pediatr Blood Cancer"},{"key":"B2","doi-asserted-by":"publisher","first-page":"2465","DOI":"10.1182\/blood.2018894618","article-title":"Recommendations for the management of hemophagocytic lymphohistiocytosis in adults.","volume":"133","author":"La Ros\u00e9e","year":"2019","journal-title":"Blood"},{"key":"B3","doi-asserted-by":"publisher","first-page":"e27929","DOI":"10.1002\/pbc.27929","article-title":"Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: recommendations 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