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

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 ad...

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Autores principales: Shen, Ziyuan, Jin, Yingliang, Sun, Qian, Zhang, Shuo, Chen, Xi, Hu, Lingling, He, Chenlu, Wang, Ying, Liu, Qinhua, Zhang, Hao, Liu, Xin, Wang, Ling, Jiao, Jun, Miao, Yuqing, Gu, Weiying, Wang, Fei, Wang, Chunling, Shi, Yuye, Ye, Jingjing, Zhu, Taigang, Sun, Cai, Song, Xuguang, Xu, Linyan, Yan, Dongmei, Sun, Haiying, Cao, Jiang, Li, Depeng, Li, Zhenyu, Wang, Zhao, Huang, Shuiping, Xu, Kailin, Sang, Wei
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894441/
https://www.ncbi.nlm.nih.gov/pubmed/35251016
http://dx.doi.org/10.3389/fimmu.2022.829878
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author Shen, Ziyuan
Jin, Yingliang
Sun, Qian
Zhang, Shuo
Chen, Xi
Hu, Lingling
He, Chenlu
Wang, Ying
Liu, Qinhua
Zhang, Hao
Liu, Xin
Wang, Ling
Jiao, Jun
Miao, Yuqing
Gu, Weiying
Wang, Fei
Wang, Chunling
Shi, Yuye
Ye, Jingjing
Zhu, Taigang
Sun, Cai
Song, Xuguang
Xu, Linyan
Yan, Dongmei
Sun, Haiying
Cao, Jiang
Li, Depeng
Li, Zhenyu
Wang, Zhao
Huang, Shuiping
Xu, Kailin
Sang, Wei
author_facet Shen, Ziyuan
Jin, Yingliang
Sun, Qian
Zhang, Shuo
Chen, Xi
Hu, Lingling
He, Chenlu
Wang, Ying
Liu, Qinhua
Zhang, Hao
Liu, Xin
Wang, Ling
Jiao, Jun
Miao, Yuqing
Gu, Weiying
Wang, Fei
Wang, Chunling
Shi, Yuye
Ye, Jingjing
Zhu, Taigang
Sun, Cai
Song, Xuguang
Xu, Linyan
Yan, Dongmei
Sun, Haiying
Cao, Jiang
Li, Depeng
Li, Zhenyu
Wang, Zhao
Huang, Shuiping
Xu, Kailin
Sang, Wei
author_sort Shen, Ziyuan
collection PubMed
description 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.
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spelling pubmed-88944412022-03-05 A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China Shen, Ziyuan Jin, Yingliang Sun, Qian Zhang, Shuo Chen, Xi Hu, Lingling He, Chenlu Wang, Ying Liu, Qinhua Zhang, Hao Liu, Xin Wang, Ling Jiao, Jun Miao, Yuqing Gu, Weiying Wang, Fei Wang, Chunling Shi, Yuye Ye, Jingjing Zhu, Taigang Sun, Cai Song, Xuguang Xu, Linyan Yan, Dongmei Sun, Haiying Cao, Jiang Li, Depeng Li, Zhenyu Wang, Zhao Huang, Shuiping Xu, Kailin Sang, Wei Front Immunol Immunology 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. Frontiers Media S.A. 2022-02-18 /pmc/articles/PMC8894441/ /pubmed/35251016 http://dx.doi.org/10.3389/fimmu.2022.829878 Text en Copyright © 2022 Shen, Jin, Sun, Zhang, Chen, Hu, He, Wang, Liu, Zhang, Liu, Wang, Jiao, Miao, Gu, Wang, Wang, Shi, Ye, Zhu, Sun, Song, Xu, Yan, Sun, Cao, Li, Li, Wang, Huang, Xu and Sang https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Shen, Ziyuan
Jin, Yingliang
Sun, Qian
Zhang, Shuo
Chen, Xi
Hu, Lingling
He, Chenlu
Wang, Ying
Liu, Qinhua
Zhang, Hao
Liu, Xin
Wang, Ling
Jiao, Jun
Miao, Yuqing
Gu, Weiying
Wang, Fei
Wang, Chunling
Shi, Yuye
Ye, Jingjing
Zhu, Taigang
Sun, Cai
Song, Xuguang
Xu, Linyan
Yan, Dongmei
Sun, Haiying
Cao, Jiang
Li, Depeng
Li, Zhenyu
Wang, Zhao
Huang, Shuiping
Xu, Kailin
Sang, Wei
A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title_full A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title_fullStr A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title_full_unstemmed A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title_short A Novel Prognostic Index Model for Adult Hemophagocytic Lymphohistiocytosis: A Multicenter Retrospective Analysis in China
title_sort novel prognostic index model for adult hemophagocytic lymphohistiocytosis: a multicenter retrospective analysis in china
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8894441/
https://www.ncbi.nlm.nih.gov/pubmed/35251016
http://dx.doi.org/10.3389/fimmu.2022.829878
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