TY - JOUR
T1 - Etiologies and Clinical Outcomes of Patients with Secondary Hemophagocytic Lymphohistiocytosis at a Tertiary PICU
AU - Dao, Dyda
AU - Xoay, Tran D.
AU - Galeano, Belinda K.
AU - Phuc, Phan H.
AU - Ouellette, Yves
N1 - Publisher Copyright:
© 2019 by the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies.
PY - 2019/7/1
Y1 - 2019/7/1
N2 - Objectives: To assess the etiologies and outcomes of patients with secondary hemophagocytic lymphohistiocytosis in the PICU. Design: Prospective observational cohort study. Setting: A single PICU at a pediatric tertiary hospital in Hanoi, Vietnam. Patients: Pediatric patients meeting the criteria for secondary hemophagocytic lymphohistiocytosis. Interventions: None. Measurements and Main Results: Between June 2017 and May 2018, 25 consecutive patients with a mean (sd) age of 23.3 months (21.6 mo) were included. Collected variables included etiologies of hemophagocytic lymphohistiocytosis and clinical and laboratory findings at admission. The Pediatric Index of Mortality 2 score at admission was calculated. Outcomes were death and multiple organ dysfunction. The severity of multiple organ dysfunction was assessed by the Pediatric Logistic Organ Dysfunction 2 score. The mean (sd) Pediatric Index of Mortality 2 predicted mortality rate was 5.6% (7.6%). Cytomegalovirus and Epstein-Barr virus coinfections (60%) were the most common suspected etiology of hemophagocytic lymphohistiocytosis. Other etiologies included Epstein-Barr virus sole infections (20%), cytomegalovirus sole infections (16%), and one unknown cause (4%). Multiple organ dysfunction (excluding hematologic failure) was found in 22 patients (88%) with death occurring in 14 patients (56%). The mean (sd) Pediatric Logistic Organ Dysfunction 2 predicted mortality rate among patients with multiple organ dysfunction was 11.9% (11.2%). Despite having lower Pediatric Index of Mortality 2 predicted mortality rates at admission, Epstein-Barr virus-cytomegalovirus coinfection cases with multiple organ dysfunction had slightly greater Pediatric Logistic Organ Dysfunction 2 predicted mortality rates than Epstein-Barr virus sole infection cases with multiple organ dysfunction: 12.2% (10.5%) versus 11.3% (11.0%). However, these rates were lower than cytomegalovirus sole infection cases with multiple organ dysfunction (14.4% [16.3%]). Area under the curve values for Pediatric Index of Mortality 2 and Pediatric Logistic Organ Dysfunction 2 were 0.74 (95% CI, 0.52-0.95) and 0.78 (95% CI, 0.52-1.00), respectively, suggesting that both scales were fair to good at predicting mortality. Conclusions: Viral infections, particularly Epstein-Barr virus-cytomegalovirus coinfections, were a common cause of secondary hemophagocytic lymphohistiocytosis. The implication of these coinfections on the clinical course of hemophagocytic lymphohistiocytosis needs to be delineated.
AB - Objectives: To assess the etiologies and outcomes of patients with secondary hemophagocytic lymphohistiocytosis in the PICU. Design: Prospective observational cohort study. Setting: A single PICU at a pediatric tertiary hospital in Hanoi, Vietnam. Patients: Pediatric patients meeting the criteria for secondary hemophagocytic lymphohistiocytosis. Interventions: None. Measurements and Main Results: Between June 2017 and May 2018, 25 consecutive patients with a mean (sd) age of 23.3 months (21.6 mo) were included. Collected variables included etiologies of hemophagocytic lymphohistiocytosis and clinical and laboratory findings at admission. The Pediatric Index of Mortality 2 score at admission was calculated. Outcomes were death and multiple organ dysfunction. The severity of multiple organ dysfunction was assessed by the Pediatric Logistic Organ Dysfunction 2 score. The mean (sd) Pediatric Index of Mortality 2 predicted mortality rate was 5.6% (7.6%). Cytomegalovirus and Epstein-Barr virus coinfections (60%) were the most common suspected etiology of hemophagocytic lymphohistiocytosis. Other etiologies included Epstein-Barr virus sole infections (20%), cytomegalovirus sole infections (16%), and one unknown cause (4%). Multiple organ dysfunction (excluding hematologic failure) was found in 22 patients (88%) with death occurring in 14 patients (56%). The mean (sd) Pediatric Logistic Organ Dysfunction 2 predicted mortality rate among patients with multiple organ dysfunction was 11.9% (11.2%). Despite having lower Pediatric Index of Mortality 2 predicted mortality rates at admission, Epstein-Barr virus-cytomegalovirus coinfection cases with multiple organ dysfunction had slightly greater Pediatric Logistic Organ Dysfunction 2 predicted mortality rates than Epstein-Barr virus sole infection cases with multiple organ dysfunction: 12.2% (10.5%) versus 11.3% (11.0%). However, these rates were lower than cytomegalovirus sole infection cases with multiple organ dysfunction (14.4% [16.3%]). Area under the curve values for Pediatric Index of Mortality 2 and Pediatric Logistic Organ Dysfunction 2 were 0.74 (95% CI, 0.52-0.95) and 0.78 (95% CI, 0.52-1.00), respectively, suggesting that both scales were fair to good at predicting mortality. Conclusions: Viral infections, particularly Epstein-Barr virus-cytomegalovirus coinfections, were a common cause of secondary hemophagocytic lymphohistiocytosis. The implication of these coinfections on the clinical course of hemophagocytic lymphohistiocytosis needs to be delineated.
KW - Epstein-Barr virus
KW - cytomegalovirus
KW - hemophagocytic lymphohistiocytosis
KW - mortality
KW - multiple organ dysfunction
KW - pediatric intensive care unit
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U2 - 10.1097/PCC.0000000000001980
DO - 10.1097/PCC.0000000000001980
M3 - Article
C2 - 31149968
AN - SCOPUS:85069272364
SN - 1529-7535
VL - 20
SP - e311-e318
JO - Pediatric Critical Care Medicine
JF - Pediatric Critical Care Medicine
IS - 7
ER -