Abstract
Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare and severe disease with a poor prognosis. We aimed to determine if 18F-fluorodeoxyglucose (18F-FDG) PET/CT-derived radiomic features alone or combination with clinical parameters could predict survival in adult HLH.Methods: This study included 70 adults with HLH (training cohort, n = 50; validation cohort, n = 20) who underwent pretherapeutic 18F-FDG PET/CT scans between August 2016 and June 2020. Radiomic features were extracted from the liver and spleen on CT and PET images. For evaluation of 6-month survival, the features exhibiting p < 0.1 in the univariate analysis between non-survivors and survivors were selected. The least absolute shrinkage and selection operator (LASSO) regression analysis was used to develop a radiomics score (Rad-score). A nomogram was built by the multivariate regression analysis to visualize the predictive model for 3-month, 6-month, and 1-year survival, while the performance and usefulness of the model were evaluated by calibration curves, the receiver operating characteristic (ROC) curves, and decision curves.Results: The Rad-score was able to predict 6-month survival in adult HLH, with area under the ROC curves (AUCs) of 0.927 (95% CI: 0.878–0.974) and 0.869 (95% CI: 0.697–1.000) in the training and validation cohorts, respectively. The radiomics nomogram combining the Rad-score with the clinical parameters resulted in better performance for predicting 6-month survival than the clinical model or the Rad-score alone. Moreover, the nomogram displayed superior discrimination, calibration, and clinical usefulness in both the cohorts.Conclusion: The newly developed Rad-score is a powerful predictor for overall survival (OS) in adults with HLH. The nomogram has great potential for predicting 3-month, 6-month, and 1-year survival, which may timely guide personalized treatments for adult HLH.
Highlights
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation characterized by hyperactive cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages leading to cytokine storm and immunemediated multiple organ failure [1, 2]
The first aim of this study was to establish a PET/CT radiomics score (Rad-score) for predicting 6-month survival in adult HLH and the second aim was to combine the Rad-score with clinical parameters, in order to develop a nomogram for predicting individual prognosis accurately and reliably
The novel prognostic factors and predictive models associated with 6-month survival in adult patients with HLH are reported via pretherapeutic 18F-FDG PET/CT radiomics analysis
Summary
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation characterized by hyperactive cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages leading to cytokine storm and immunemediated multiple organ failure [1, 2]. HLH has been classified as primary or familial HLH driven by underlying genetic defects in cytotoxic immune function or as secondary or reactive HLH caused by infections [e.g., EpsteinBarr virus (EBV), cytomegalovirus (CMV), HIV, and coronavirus disease 2019 (COVID-19)], malignancies (e.g., hematologic malignancies), and autoimmune diseases (e.g., macrophage activation syndrome) [1]. Because of few data and/or no prospective studies for adult HLH, pediatric data are often generalized to guide diagnostic, therapeutic, and prognostic decision-making in adults [1, 6]. Identifying poor prognosis in adult HLH is crucial for risk stratification and therapeutic decision-making. It would be of great utility to build a predictive model to precisely evaluate the prognosis in adult HLH based on multiple indictors
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