Hemophagocytic Lymphohistiocytosis Patients Need Intensive Care Can be Successfully Rescued By Timely Using Etoposide-Based HLH Regimens

Abstract

Objectives:Hemophagocytic lymphohistiocytosis (HLH) patients who need life support and intensive care usually have multiple organ failure and high mortality. However, the clinical characteristics, therapeutic efficacy and outcome in these critically ill HLH patients have remained unclear. Methods: In the study, we analyzed the data of 41 critically ill HLH patients treated in a prospective, non-randomized clinical trial (ChiCTR1800015283). Results: 41 secondary HLH patients need life support and intensive care, the median age was 47 years (9-79 years), 22 males and 19 females. The median sequential organ failure assessment (SOFA) score at diagnosis was 8 (4-17). 70.73% of critically ill HLH patients had septic shock, followed by disseminated intravascular coagulation (DIC, 58.54%) , acute respiratory distress syndrome (53.66%). 22 patients (53.66%) needed invasive or non-invasive positive pressure mechanical ventilation, 26 patients (63.41%) needed vasoactive drugs, and 4 patients (9.76%) needed continuous renal replacement therapy (CRRT). Among 10 patients treated with DEP regimen, the median time to remove ECG monitoring, respiratory support, stop using vasoactive drugs, converting from dominant DIC to non dominant DIC was respectively 8.5 days, 8 days, 6.5 days and 4 days. After 4 weeks, 5 patients achieved PR, and 3 patients were evaluated as NR. However, among 4 patients treated with HLH-94/2004, the median time to remove ECG monitoring, respiratory support, converting from dominant DIC to non dominant DIC was respectively 30 days, 14 days and 4 days. After 4 weeks, 3 patients were evaluated as NR. Up to the last follow-up, the OS rate of 14 patients receiving DEP/ HLH-94/2004 regimens was 78.6%. In contrast, all 27 untreated HLH patients died. Univariate analysis showed that PCT>0.5 ug/l, respiratory failure, renal failure and liver failure were the negative factors affecting survival (p=0.018, 0.003, 0.001, 0.006). Multivariate analysis showed that respiratory failure (P=0.014), renal failure (P=0.019) and liver failure(P=0.049) were independent adverse factors. Conclusion: HLH patients need intensive care timely used etoposide-based HLH regimens especially DEP regimen which might successfully rescue the critically ill patients.