A novel prognostic model for adult patients with Hemophagocytic Lymphohistiocytosis

Abstract

Background: 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. Results: Three 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. Conclusion: These 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.

Development and Validation of a Nomogram Based on 18F-FDG PET/CT Radiomics to Predict the Overall Survival in Adult Hemophagocytic Lymphohistiocytosis

“…High ALT and AST/ALT ratio have been found to act as adverse prognostic factors in adult HLH (8,28). Furthermore, hepatic involvement and hepatomegaly reveal poor prognostic indicators and early death predictors in HLH (8,28). In line with these studies, our results illustrated that the radiomic features of spleen and liver presented great prognostic values for adult HLH.…”

“…In addition, the well-known liver enzymes, AST and ALT, are identified as indicators of various diseases including HLH. High ALT and AST/ALT ratio have been found to act as adverse prognostic factors in adult HLH (8,28). Furthermore, hepatic involvement and hepatomegaly reveal poor prognostic indicators and early death predictors in HLH (8,28).…”

“…Recent studies pointed out that a number of clinical parameters play a role in the prognosis of HLH such as lymphoid malignancy, hemoglobin, platelets, CRP, and cytopenia [ (8,9,50,51)]. It is well-documented that lymphoid malignancy is negatively associated with survival.…”

“…Therefore, identifying poor prognosis in adult HLH is crucial for risk stratification and therapeutic decision-making. Recently, it has been reported that clinical and laboratory markers are correlated with survival in adult HLH including age, platelet, fibrinogen, albumin, serum ferritin, alanine aminotransferase (ALT), and malignancy (1,2,8,9). But none of them can be a single effective prognostic factor as a result of poor sensitivity and/or specificity.…”

A Decade of Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: Does the Outcome Improve?

“…Various studies have shown different prognostic factors for adult HLH. Hypofibrinogenemia, thrombocytopenia, hypoalbuminemia, hyperferritinemia, and elevated lactate dehydrogenase levels are significant predictors of inferior survival [ 18 , 19 ]. Regardless of these identified prognostic factors, tumor-associated HLH had a markedly worse outcome, emphasizing the importance of underlying disease control in lymphoma-associated HLH.…”