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

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 alone or concurrent lymphoma-associated phagocytic syndrome (LAHS) admitted to Beijing Friendship Hospital from January 2016 to December 2020 were enrolled in this study. FINDINGS: There 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. CONCLUSION: LAHS 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.