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
Prevalence and mortality of haemophagocytic lymphohistiocytosis in dengue fever: a systematic review and meta-analysis Leong Tung Ong, Roovam Balasubramaniam Transactions of The Royal Society of Tropical Medicine and Hygiene.2024;[Epub] CrossRef
Predicting relapsed/refractory disease in childhood hemophagocytic lymphohistiocytosis based on clinical features at diagnosis: A 13-year single-institute retrospective study in Thailand Pattranan Kusontammarat, Chane Choed-Amphai, Lalita Sathitsamitphong, Watchareewan Sontichai, Rungrote Natesirinilkul, Pimlak Charoenkwan Annals of Hematology.2024;[Epub] CrossRef
Autoimmune encephalitis followed by hemophagocytic lymph histiocytosis: a case report Li Huang, Jie Tan, Peihao Lin, Zixuan Chen, Qihua Huang, Haiyan Yao, Lihong Jiang, Baoyi Long, Youming Long Frontiers in Immunology.2024;[Epub] CrossRef
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