Journal of Yeungnam Medical Science

Original article

A retrospective analysis of etiology and outcomes of hemophagocytic lymphohistiocytosis in children and adults: Abraham Kwak, Nani Jung, Ye Jee Shim, Heung Sik Kim, Hyun Ji Lim, Jae Min Lee, Mi Hwa Heo, Young Rok Do; Yeungnam Univ J Med. 2021;38(3):208-218. Published online November 27, 2020; DOI: https://doi.org/10.12701/yujm.2020.00591

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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.

Citations

Citations to this article as recorded by

Spontaneous resolution of hemophagocytic lymphohistiocytosis in a child infected with epstein–Barr virus
Rita Alfattal, Hussain Sadeq, Abdullah Ali
Journal of Applied Hematology.2023; 14(1): 57. CrossRef
Pediatric inborn errors of immunity causing hemophagocytic lymphohistiocytosis: Case report and review of the literature
María Soledad Caldirola, Andrea Gómez Raccio, Daniela Di Giovanni, María Isabel Gaillard, María Victoria Preciado
Journal of Leukocyte Biology.2022; 112(4): 607. CrossRef
Secondary Hemophagocytic Lymphohistiocytosis and Autoimmune Cytopenias: Case Description and Review of the Literature
Bruno Fattizzo, Marta Ferraresi, Juri Giannotta, Wilma Barcellini
Journal of Clinical Medicine.2021; 10(4): 870. CrossRef
HLH-Like Syndrome and Rhabdomyolysis in an Adolescent Patient
Lauren T. Maloney, Bronwyn Baz, Dia Hazra
Pediatrics.2021;[Epub] CrossRef

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