A novel prognostic model for adult patients with Hemophagocytic Lymphohistiocytosis

Hua-Guo Xu,Jing Zhou,Zhi-Qi Wu,Hemant Goyal,Jun Zhou

doi:10.1186/s13023-020-01496-4

Abstract

BackgroundHemophagocytic Lymphohistiocytosis (HLH) is a type of rare disease with low survival rate. We aimed to develop a model to evaluate the six-month prognosis in adult HLH patients. The data at discharge (will be called as post-treatment) for newly diagnosed adult HLH patients was collected and independent prognostic variables were selected for inclusion in the model.ResultsThree laboratory markers were confirmed to be the independent risk factors (ferritin: hazard ratio (HR) 0.101, 95% confidence interval (CI) 0.036–0.282, P<0.001; platelets: HR 4.799, 95% CI 1.884–12.223, P = 0.001; alanine aminotransferase (ALT): HR 0.423, 95% CI 0.180–0.997, P = 0.049). These were included in the final clinical prediction model. Receiver operating characteristic (ROC) curves disclosed that this model had a better discrimination (area under the curve (AUC) = 0.842, 95% CI 0.773–0.910, P < 0.001) than each of them alone and the calibration curves aligned completely with the model predictions and actual observations. Kaplan-Meier curves revealed a significant difference in the overall survival (OS) in patients stratified by the model with higher values associated with a better OS.ConclusionThese results point out that serum ferritin, platelets and ALT levels are independent elements of OS in adult patients with HLH, and that the proposed model have a better prognostic value than any of these markers alone.

Highlights

Hemophagocytic Lymphohistiocytosis (HLH) is a type of rare disease with low survival rate
The results indicated that patients’ ferritin, neutrophils, HB, platelets, alanine aminotransferase (ALT), Aspartate aminotransferase (AST), Lactate dehydrogenase (LDH), αhydroxybutyrate dehydrogenase (α-HBDH), Direct bilirubin (DBIL), albumin, Urea nitrogen (UREA), CREA, Uric acid (UA) and Ca2+ were associated with the six-month prognosis in HLH patients
The results showed that the area-under-the curve (AUC) values of the model was 0.842 (Fig. 2), which was obviously superior than those of ferritin, platelets and Alanine aminotransferase (ALT) in cohort

Summary

Introduction

Hemophagocytic Lymphohistiocytosis (HLH) is a type of rare disease with low survival rate. We aimed to develop a model to evaluate the six-month prognosis in adult HLH patients. The data at discharge (will be called as post-treatment) for newly diagnosed adult HLH patients was collected and independent prognostic variables were selected for inclusion in the model. Hemophagocytic Lymphohistiocytosis (HLH) is a rare invariable fatal disease (if untreated) accompanied with the secretion of a huge number of cytokines which trigger a series of chain inflammatory reactions [1, 2]. Greater number of adult HLH cases are being diagnosed and reported because of increasing recognition and better management [12]. The adult HLH progression is usually complicated, sometimes changeable and the mortality rate varies considerably from 8 to 60% [14,15,16,17].

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