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논문 기본 정보

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학술저널
저자정보
Abraham Kwak (Department of Pediatrics Keimyung University Dongsan Hospital Keimyung University School of Medicin) Nani Jung (Department of Pediatrics Keimyung University Dongsan Hospital Keimyung University School of Medicin) Ye Jee Shim (Department of Pediatrics Keimyung University Dongsan Hospital Keimyung University School of Medicin) Heung Sik Kim (Department of Pediatrics Keimyung University Daegu Dongsan Hospital Daegu Korea) Hyun Ji Lim (Department of Pediatrics Yeungnam University Hospital Daegu Korea) Jae Min Lee (Department of Pediatrics Yeungnam University College of Medicine Daegu Korea) Mi Hwa Heo (Department of Internal Medicine Keimyung University Dongsan Hospital Keimyung University School of) Young Rok Do (Department of Internal Medicine Keimyung University Dongsan Hospital Keimyung University School of)
저널정보
영남대학교 의과대학 Journal of Yeungnam Medical Science Yeungnam University Journal of Medicine 제38권 제3호
발행연도
2021.1
수록면
208 - 218 (11page)

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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.
#Human herpesvirus 4 #Hemophagocytic lymphohistiocytosis #Lymphoproliferative disorders #Neoplasms #Survival

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