Yeungnam Univ J Med.  2021 Jul;38(3):208-218. 10.12701/yujm.2020.00591.

A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults

Affiliations
  • 1Department of Pediatrics, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Korea
  • 2Department of Pediatrics, Keimyung University Daegu Dongsan Hospital, Daegu, Korea
  • 3Department of Pediatrics, Yeungnam University Hospital, Daegu, Korea
  • 4Department of Pediatrics, Yeungnam University College of Medicine, Daegu, Korea
  • 5Department of Internal Medicine, Keimyung University Dongsan Hospital, Keimyung University School of Medicine, Daegu, Korea

Abstract

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.

Keyword

Epstein-Barr virus; Hemophagocytic lymphohistiocytosis; Neoplasms; Survival; X-linked lymphoproliferative disease
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