{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T02:34:14Z","timestamp":1760236454824,"version":"build-2065373602"},"reference-count":30,"publisher":"MDPI AG","issue":"12","license":[{"start":{"date-parts":[[2021,11,24]],"date-time":"2021-11-24T00:00:00Z","timestamp":1637712000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"the Korea Health Technology R&amp;D Project through the Korea Health Industry Development In-stitute (KHIDI), funded by the Ministry of Health &amp; Welfare, Republic of Korea","award":["HI18C0998"],"award-info":[{"award-number":["HI18C0998"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Children"],"abstract":"<jats:p>It is complicated to establish a consensus on the management and diagnosis of malignancy-triggered hemophagocytic lymphohistiocytosis (M-HLH) in children, as an initial presentation of malignancy is complicated. In this paper, we analyze the clinical characteristics and outcomes of eight pediatric patients in which M-HLH was the initial presentation of malignancy. All patients had hematologic malignancies: three subcutaneous panniculitis-like T-cell lymphomas, two acute lymphoblastic leukemias, two anaplastic large cell lymphomas, and a systemic EBV + T-cell lymphoma of childhood. The incidence rate of M-HLH among leukemia and malignant lymphoma patients in our institution was 1.9%. From the initial diagnosis of HLH, the median time taken to be diagnosed as a malignancy was about 1.3 months. The majority of patients received HLH-targeted immunosuppression and\/or etoposide at first. The patients\u2019 clinical response to treatment for HLH and malignancies were varied. Five out of the eight patients died, one of whom died due to HLH-related cerebral edema after the initiation of chemotherapy. The median overall survival was 1.6 years. In order to improve the survival rate, the early detection of M-HLH, rapid screening for malignancy, and complete control of M-HLH with HLH-directed therapy followed by a thorough response monitoring are required.<\/jats:p>","DOI":"10.3390\/children8121083","type":"journal-article","created":{"date-parts":[[2021,11,28]],"date-time":"2021-11-28T22:19:16Z","timestamp":1638137956000},"page":"1083","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Hemophagocytic Lymphohistiocytosis as Initial Presentation of Malignancy in Pediatric Patients: Rare but Not to Be Ignored"],"prefix":"10.3390","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9103-8631","authenticated-orcid":false,"given":"Hye-ji","family":"Han","sequence":"first","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8822-1988","authenticated-orcid":false,"given":"Kyung Taek","family":"Hong","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyun Jin","family":"Park","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bo Kyung","family":"Kim","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hong Yul","family":"An","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jung Yoon","family":"Choi","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hyoung Jin","family":"Kang","sequence":"additional","affiliation":[{"name":"Departments of Pediatrics, Seoul National University College of Medicine, Seoul 03080, Korea"},{"name":"Cancer Research Institute, Seoul National University, Seoul 03080, Korea"},{"name":"Wide River Institute of Immunology, Hongcheon 25159, Korea"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2021,11,24]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","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. 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