Prognostic Factors of Early Death in Childhood Hemophagocytic Lymphohistiocytosis: Experience From A Single Tertiary Center in Thailand
DOI:
https://doi.org/10.31584/jhsmr.2022880Keywords:
hemophagocytic, lymphohistiocytosis, prognostic factors, survival outcomeAbstract
Objective: To establish the clinical profile, outcomes, and risk factors of mortality in pediatric hemophagocytic lymphohistiocytosis (HLH) patients admitted to a tertiary care hospital in the south of Thailand.
Material and Methods: The medical records of HLH patients aged under 15 years were retrospectively reviewed. Survival times were estimated using the Kaplan-Meier estimator. Univariate associations between covariates and survival were visualized with graphs and compared using the log-rank test. Factors with statistical significance in the univariate analysis were included in a multivariate cox regression analysis.
Results: A total of 24 childhood HLH cases were identified over the 20-year study period. Central nervous system (CNS) involvement at diagnosis (hazard ratio [HR]: 7.7, 95% CI: 1.2-51.2), absolute neutrophil count (ANC) <1,000.0 cells/μL (HR: 33.3, 95% CI: 2.7-39.8) and renal insufficiency (HR: 28.2, 95% CI: 2.1-373.8) were adverse risk factors. Forty-six percent of the children survived at least 30 days after diagnosis with a median survival time of 21 days.
Conclusion: CNS involvement, low ANC and renal insufficiency at diagnosis were adverse risk factors for early death in HLH children.
References
Bin Q, Gao JH, Luo JM. Prognostic factors of early outcome in pediatric hemophagocytic lymphohistiocytosis: an analysis of 116 cases. Ann Hematol 2016;95:1411-8.
Jordan MB, Allen CE, Greenberg J, Henry M, Hermiston ML, Kumar A, et al. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO). Pediatr Blood Cancer 2019;66:e27929.
Simon AC, Delhi CG, Basu D, Ramesh KR. Hemophagocytic lymphohistiocytosis in children: clinical profile and outcome. J Pediatr Hematol Oncol 2020;42:e281-5.
Henter JI, Horne A, Arico M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31.
Luo ZB, Chen YY, Xu XJ, Zhao N, Tang YM. Prognostic factors of early death in children with hemophagocytic lymphohistiocytosis. Cytokine 2017;97:80-5.
Kim MM, Yum MS, Choi HW, Ko TS, Im HJ, Seo JJ, et al. Central nervous system (CNS) involvement is a critical prognostic factor for hemophagocytic lymphohistiocytosis. Korean J Hematol 2012;47:273-80.
Xu XJ, Wang HS, Ju XL, Xiao PF, Xiao Y, Xue HM, et al. Clinical presentation and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in China: A retrospective multicenter study. Pediatr Blood Cancer 2017;64:e26264.
Chen TY, Hsu MH, Kuo HC, Sheen JM, Cheng MC, Lin YJ. Outcome analysis of pediatric hemophagocytic lymphohistiocytosis. J Formos Med Assoc 2021;120:172-9.
Trottestam H, Horne A, Arico M, Egeler RM, Filipovich AH, Gadner H, et al. Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol. Blood 2011;118:4577-84.
Schwartz GJ, Haycock GB, Edelmann CM, Spitzer A. A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 1976;58:259-63.
Haddad E, Sulis M, Jabado N, Blanche S, Fischer S, Tardieu M. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood 1997;89:794–800.
Zhao YZ, Zhang Q, Li ZG, Zhang L, Lian HY, Ma HH, et al. Central nervous system involvement in 179 Chinese children with hemophagocytic lymphohistiocytosis. Chin Med J 2018; 131:1786-92.
Xue H, Chen C, Li W, Lin C, Fang J, Li Y, et al. Analysis of prognostic risk factors in children with Epstein-Barr virus associated hemophagocytic syndrome. Minerva Pediatr 2015;67:251-61
Yanagisawa R, Nakazawa Y, Matsuda K, Yasumi T, Kanegane H, Ohga S, et al. Outcomes in children with hemophagocytic lymphohistiocytosis treated using HLH-2004 protocol in Japan. Int J Hematol 2019;109:206-13.
Koh KN, Im HJ, Chung NG, Cho B, Kang HJ, Shin HY, et al. Clinical features, genetics, and outcome of pediatric patients with hemophagocytic lymphohistiocytosis in Korea: report of a nationwide survey from Korea histiocytosis working party. Eur J Haematol 2015;94:51-9.
Kaya Z, Bay A, Albayrak M, Kocak U, Yenicesu I, Gursel T. Prognostic factors and long-term outcome in 52 Turkish children with hemophagocytic lymphohistiocytosis. Pediatr Crit Care Med 2015;16:165-73.
Wang YR, Qiu YN, Bai Y, Wang XF. A retrospective analysis of 56 children with hemophagocytic lymphohistiocytosis. J Blood Med 2016;7:227-31.
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