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

Abstract

Background: Malignancies, especially lymphoma, are a common cause of adult secondary HLH and an independent risk factor for the prognosis of HLH patients. Methods: Patients with lymphoma or lymphoma-associated phagocytic syndrome (LAHS) admitted to Beijing Friendship Hospital from January 2016 to December 2020 were enrolled in this study. Findings: In total, there were 348 lymphoma patients, 104 with LAHS and 244 without LAHS. The pathological type of non-LAHS was dominated by B-cell lymphoma, while LAHS was T/NK-cell lymphoma predominantly (p <0.001). Superficial lymph node enlargement was more significant in patients with B-LAHS compared with T/NK-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 (p <0.001), and were more likely to have 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, platelets count, IPI, history of NK/T lymphoma and no remission of HLH were independent risk factors for poor prognosis in patients with non-Hodgkin lymphoma associated phagocytic syndrome (NHL-LAHS), and a prognostic risk score model for NHL-LAHS was developed. Interpretation: LAHS is a life-threatening disease with poor prognosis. The prognostic risk score model for NHL-LAHS developed with a good fit and good validation for test has value for clinical application. Funding: Beijing Municipal Administration of Hospital Clinical Medicine Development of Special Funding Support (XMLX201823); National Natural Science Foundation of China (81871633); Beijing Natural Science Foundation (7181003); Beijing Municipal Administration of Hospitals’ Ascent Plan (DFL20180101). Declaration of Interest: None to declare. Ethical Approval: It was approved by the Ethics Committee at Beijing Friendship Hospital