Clinical Features and Prognostic Risk Prediction of Non-Hodgkin Lymphoma-Associated Hemophagocytic Syndrome.

Shuyan Yao,Ruoxi Zhang,Lingbo He,Zhao Wang,Zhengjie Hua,Menghan Liu,Yini Wang,Zhili Jin

doi:10.3389/fonc.2021.788056

Abstract

BackgroundMalignancies, especially lymphoma, are a common cause of adult secondary HLH and an independent risk factor for the prognosis of HLH patients.MethodsPatients with lymphoma alone or concurrent lymphoma-associated phagocytic syndrome (LAHS) admitted to Beijing Friendship Hospital from January 2016 to December 2020 were enrolled in this study.FindingsThere were 348 lymphoma patients, 104 concurrent with LAHS. The pathological type of lymphoma without LAHS was dominated by B-cell lymphoma, while those with LAHS were T/NK-cell lymphoma predominantly (p < 0.001). Superficial lymph node enlargement was more significant in patients with B-LAHS (p = 0.006), while patients with T/NK-LAHS had lower neutrophil counts (p = 0.005), lower fibrinogen levels (p < 0.001), higher transaminase levels, and more co-infection with EBV (p < 0.001). B-LAHS had significantly higher IL-10 levels than with T/NK-LAHS (p = 0.006), and NK/T-LAHS had significantly higher IP-10 levels than other T-LAHS (p = 0.008). Age, platelet count, IPI, history of NK/T lymphoma, and no remission of HLH were independent risk factors for prognosis in patients with non-Hodgkin lymphoma-associated phagocytic syndrome (NHL-LAHS), and a prognostic risk score model for NHL-LAHS was developed.ConclusionLAHS is a life-threatening disease with a poor prognosis. The prognostic risk score model for NHL-LAHS with a good fit and validation for the test has value for clinical application.

Highlights

Hemophagocytic syndrome (HPS), known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome resulting from inherited or acquired abnormalities of immune function [1]
Patients with lymphoma alone or concurrent lymphomaassociated phagocytic syndromes (LAHS) admitted to Beijing Friendship Hospital from January 2016 to December 2020 were enrolled in this study
All patients with confirmed LAHS were screened for cytotoxic functions, including natural killer (NK) cell activity and degranulation function assays, and expression of proteins corresponding to HLH-deficient genes such as perforin, granzyme B, SAP, and XIAP

Summary

Introduction

Hemophagocytic syndrome (HPS), known as hemophagocytic lymphohistiocytosis (HLH), is a clinical syndrome resulting from inherited or acquired abnormalities of immune function [1]. The multifactorial analysis identified malignancy as an independent risk factor for the. Non-Hodgkin Lymphoma-Associated Hemophagocytic Syndrome prognosis of patients with HLH, and lymphoma was the most common cause of malignancy-associated HLH [3]. Studies tended to focus on a subgroup of lymphomas, mostly NK/T lymphomas, and rarely tested the levels of soluble interleukin 2 receptors (sCD25) or the function of natural killer (NK) cells in adults [4,5,6,7]. This study aimed at demonstrating the clinical features of lymphomaassociated phagocytic syndromes (LAHS), establishing prognostic stratification, gaining a more comprehensive understanding of LAHS, and improving the overall prognosis of LAHS. Malignancies, especially lymphoma, are a common cause of adult secondary HLH and an independent risk factor for the prognosis of HLH patients

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