Journal of Yeungnam Medical Science

영남대 의대 학술지 편집위원회 (Yeungnam University College of Medicine, Yeungnam University Institute Medical Science)
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A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults

  • Kwak, Abraham (Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine) ;
  • Jung, Nani (Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine) ;
  • Shim, Ye Jee (Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine) ;
  • Kim, Heung Sik (Department of Pediatrics, Keimyung University Daegu Dongsan Hospital) ;
  • Lim, Hyun Ji (Department of Pediatrics, Yeungnam University Hospital) ;
  • Lee, Jae Min (Department of Pediatrics, Yeungnam University College of Medicine) ;
  • Heo, Mi Hwa (Department of Internal Medicine, Keimyung University Dongsan Hospital, Keimyung University School of Medicine) ;
  • Do, Young Rok (Department of Internal Medicine, Keimyung University Dongsan Hospital, Keimyung University School of Medicine)
  • 투고 : 2020.07.11
  • 심사 : 2020.09.25
  • 발행 : 2021.07.31
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초록

Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare but severe, life-threatening inflammatory condition if untreated. We aimed to investigate the etiologies, outcomes, and risk factors for death in children and adults with HLH. Methods: The medical records of patients who met the HLH criteria of two regional university hospitals in Korea between January 2001 and December 2019 were retrospectively investigated. Results: Sixty patients with HLH (35 children and 25 adults) were included. The median age at diagnosis was 7.0 years (range, 0.1-83 years), and the median follow-up duration was 8.5 months (range, 0-204 months). Four patients had primary HLH, 48 patients had secondary HLH (20 infection-associated, 18 neoplasm-associated, and 10 autoimmune-associated HLH), and eight patients had HLH of unknown cause. Infection was the most common cause in children (14/35, 40.0%), whereas neoplasia was the most common cause in adults (13/25, 52.0%). Twenty-eight patients were treated with HLH-2004/94 immunochemotherapy. The 5-year overall survival (OS) rate for all HLH patients was 59.9%. The 5-year OS rates for patients with primary, infection-associated, neoplasm-associated, autoimmune-associated, and unknown cause HLH were 25.0%, 85.0%, 26.7%, 87.5%, and 62.5%, respectively. Using multivariate analysis, neoplasm-induced HLH (p=0.001) and a platelet count <50×109/L (p=0.008) were identified as independent risk factors for poor prognosis in patients with HLH. Conclusion: Infection was the most common cause of HLH in children, while it was neoplasia in adults. The 5-year OS rate for all HLH patients was 59.9%. HLH caused by an underlying neoplasm or a low platelet count at the time of diagnosis were risk factors for poor prognosis.

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참고문헌

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피인용 문헌

  1. Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature vol.10, pp.4, 2021, https://doi.org/10.3390/jcm10040870