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A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China

Authors :
Ziyuan Shen
Yingliang Jin
Qian Sun
Shuo Zhang
Xi Chen
Lingling Hu
Chenlu He
Ying Wang
Qinhua Liu
Hao Zhang
Xin Liu
Ling Wang
Jun Jiao
Yuqing Miao
Weiying Gu
Fei Wang
Chunling Wang
Yuye Shi
Jingjing Ye
Taigang Zhu
Cai Sun
Xuguang Song
Linyan Xu
Dongmei Yan
Haiying Sun
Jiang Cao
Depeng Li
Zhenyu Li
Zhao Wang
Shuiping Huang
Kailin Xu
Wei Sang
Source :
Frontiers in Immunology, Vol 13 (2022)
Publication Year :
2021

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an immune disorder with rapid progression and poor survival. Individual treatment strategy is restricted, due to the absence of precise stratification criteria. In this multicenter retrospective study, we aimed to develop a feasible prognostic model for adult HLH in China. A total of 270 newly diagnosed patients of adult HLH were retrieved from the Huaihai Lymphoma Working Group (HHLWG), of whom 184 from 5 medical centers served as derivation cohort, and 86 cases from 3 other centers served as validation cohort. X-Tile program and Maxstat analysis were used to identify optimal cutoff points of continuous variables; univariate and multivariate Cox analyses were used for variable selection, and the Kaplan–Meier curve was used to analyze the value of variables on prognosis. The C-index, Brier Score, and calibration curve were used for model validation. Multivariate analysis showed that age, creatinine, albumin, platelet, lymphocyte ratio, and alanine aminotransferase were independent prognostic factors. By rounding up the hazard ratios from 6 significant variables, a maximum of 9 points was assigned. The final scoring model of HHLWG-HPI was identified with four risk groups: low risk (≤3 pts), low-intermediate risk (4 pts), high-intermediate risk (5-6 pts), and high risk (≥7 pts), with 5-year overall survival rates of 68.5%, 35.2%, 21.3%, and 10.8%, respectively. The C-indexes were 0.796 and 0.758 in the derivation and validation cohorts by using a bootstrap resampling program. In conclusion, the HHLWG-HPI model provides a feasible and accurate stratification system for individualized treatment strategy in adult HLH.

Details

ISSN :
16643224
Volume :
13
Database :
OpenAIRE
Journal :
Frontiers in immunology
Accession number :
edsair.doi.dedup.....26e0175a5d27bbbf8f618145eb0cf401