Prognostic factors of early outcome in pediatric hemophagocytic lymphohistiocytosis: an analysis of 116 cases.
Early mortality remains a major challenge for the treatment of hemophagocytic lymphohistiocytosis (HLH), which warrants the need for prompt risk stratification in the early phase of the disease. We retrospectively analyzed clinical features of a cohort of pediatric patients managed at a tertiary hospital in southern China from 2005 to 2015. A total of 116 patients (median age 27.5 months) with predominantly secondary HLH were included. In a multivariate Cox regression model, neutrophils <0.5 × 10(9)/L (risk ratio (RR) = 5.01; 95 % confidence interval (CI) 1.55-16.20; P = 0.007), total bilirubin over twofold upper limit of normal value (RR = 2.86; 95 % CI 0.83-9.88; P = 0.097), and albumin ≤20 g/L (RR = 5.79; 95 % CI 1.70-19.73; P = 0.005) at diagnosis were independent risk factors for 30-day mortality. The 30-day overall survival rate (OS) of patients with three risk factors was significantly lower than that of patients with zero to two risk factors (0 vs 90.7 %; P<0.001). Patients with three risk factors were 64-fold more likely to have early adverse outcome as compared to patients with zero to two risk factors (RR = 64.45; 95 % CI 18.35-226.33; P<0.001). Platelet count normalization in 2 weeks was an independent predictor for resolution after initial therapy with an odds ratio (OR) of 18.4 (95 % CI 2.7-122.9; P = 0.003). Our results indicate that severe neutropenia and liver function damage are prognostic factors for early death in HLH and platelet count normalization in 2 weeks is a critical predictor for resolution after initial therapy.
Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Guangxi Medical University, Nanning, 530021, Guangxi, China.
Qiong
Q
Bin
Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Guangxi Medical University, Nanning, 530021, Guangxi, China.
Jin-Hong
JH
Gao
Department of Pediatrics, The First Affiliated Hospital of Guangxi Medical University, Guangxi Medical University, Nanning, 530021, Guangxi, China. luojm23@126.com.
Jian-Ming
JM
Luo
D000293:Adolescent; D001663:Bilirubin / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D053208:Kaplan-Meier Estimate; D008099:Liver / Q000473:pathology / Q000503:physiopathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000097:blood / Q000401:mortality / Q000628:therapy*; D008297:Male; D015999:Multivariate Analysis; D009503:Neutropenia / Q000097:blood; D017063:Outcome Assessment, Health Care / Q000379:methods* / Q000706:statistics & numerical data*; D010976:Platelet Count; D011379:Prognosis; D016016:Proportional Hazards Models; D012189:Retrospective Studies; D012307:Risk Factors; D012709:Serum Albumin / Q000032:analysis; D015996:Survival Rate; D013997:Time Factors
D012709:Serum Albumin; D001663:Bilirubin
Early death; Hemophagocytic lymphohistiocytosis; Outcome; Prognostic factor; Resolution