Analysis of clinical characteristics and prognostic factors of hemophagocytic lymphohistiocytosis in pediatric intensive care unit / 中华实用儿科临床杂志

ABSTRACT

Objective:To explore the clinical features and the mortality risk factors of hemophagocytic lymphohistiocytosis (HLH) in pediatric intensive care unit (PICU).Methods:Clinical data of 68 children diagnosed as HLH and treated in PICU, the Children′s Hospital, Zhejiang University School of Medicine between January 2014 and December 2020 were retrospectively analyzed.According to the lowest pediatric critical illness score (PCIS) within 24 h after admission to PICU, they were divided into non-critical group, critical group and extremely critical group.Moreover, they were further divided into survivors and non-survivors based on the prognosis.Clinical characteristics in each group were analyzed and compared. Logistic regression analysis was performed to obtain the estimates of odds ratio ( OR) and corresponding 95% confidence interval ( CI) of possible predictive factors for death. Results:The median age of recruited 68 children with HLH and treated in PICU was 26 months, involving 39 female and 29 male patients.The overall mortality rate was 45.59%(31/68 cases). Epstein-Barr virus (EBV) infection was the major cause of HLH.The mortality of non-critical group, critical group and extremely critical group were 21.05% (8/38 cases), 57.14% (8/14 cases) and 93.75% (15/16 cases), the difference was statistically significant ( P<0.05). The PCIS, the pediatric logistic organ dysfunction score 2 (PELOD-2), duration of fever, mechanical ventilation within 6 h, vasoactive use, gastrointestinal and pulmonary hemorrhage, disseminated intravascular coagulation (DIC), hepatobiliary dysfunction (HBD) and acute kidney injury (AKI), acidosis[pH and base excess (BE)], hemoglobin (Hb), prothrombin time (PT), and activated partial thromboplastin time (APTT), serum creatinine (Scr), interleukin-6 (IL-6) were significantly different between survivors and non-survivors (all P<0.05). The Logistic regression analysis showed that PCIS ( OR=0.800, 95% CI 0.707-0.905, P<0.001), Hb ( OR=0.929, 95% CI 0.871-0.991, P=0.027), APTT ( OR=0.954, 95% CI 0.910-0.990, P=0.047), and AKI( OR=29.064, 95% CI 3.072-274.957, P=0.003) were risk factors for the death of HLH. Conclusions:HLH has a very high mortality and requires critical care in PICU, low PCIS, anemia, prolonged APTT and AKI are independent risk factors for the death of HLH.