Clinical Characteristics And Prognostic Analysis Of Lymphoma Associated Hemophagocytic Lymphohistiocytosis

Objective:Retrospective analysis of the Clinical characteristics,treatment and prognostic analysis of lymphoma associated hemophagocytic lymphohistiocytosis(LA-HLH).Methods:The clinical data of patients diagnosed with LA-HLH in the Second Hospital of Shanxi Medical University from September 2016 to September 2022 were collected,and the survival curve was maked by Kaplan-Meier method,the Log-rank test was used for comparison,and multivariate analysis by Cox proportional hazards regression model.Results:The 44 patients with LA-HLH median age was 59 years,which were more common in male(59.0%).The common type of lymphoma pathology was diffuse large B-cell lymphoma(n=17;38.6%),and HLH occurred before or at the same time as lymphoma diagnosis in 34 patients(77.3%).The clinical manifestations of LA-HLH are fever(100.0%)and lymphadenopathy(100.0%);Laboratory abnormalities were common as hyperferritinemia(100.0%),bone marrow phagocytosis(91.0%),and hyperlactate dehydrogenase(88.7%).Compared with B-LA-HLH,NK/T-LA-HLH had a slight difference in the red blood cell count(P=0.043),hemoglobin(P=0.014),activated partial thromboplastin time(P=0.046),serum aspartate aminotransferase(P=0.027),triglycerides(P=0.027),peripheral blood EBV DNA load(P<0.001),and EBER positivity rate in tissues(P= 0.001)is higher than the former;Fibrinogen(P=0.034)was lower than that of the former,and there was a statistical difference.The median survival time for LA-HLH was 157 days,with NK/T-LA-HLH patients having the shortest median survival(P=0.024).Patients initially treated with antilymphoma had better overall survival(OS)than other regimens(P=0.046)and objective response rate(0RR)than other regimens(P>0.05).Age≥ 70 years(P=0.039),hepatomegaly(P=0.037),ferritin ≥1500ug/L(P=0.037),high s CD25(P=0.005),high IL-10(P=0.009),high IFN-γ(P=0.033),and decreased Th/Ts ratio(P=0.048)were independent prognostic factors for adverse outcomes in patients with LAHLH.High EBV DNA load(P=0.025)was highly correlated with adverse outcomes in patients with HLH associated with B-cell lymphoma.Conclusion:LA-HLH can occur in the initial/recurrent or maintenance treatment phase of lymphoma,which lacks specificity,rapid progression,and poor prognosis,with NK/T-cell lymphoma-associated HLH having the worst prognosis.Initiation of anti-lymphoma therapy can help improve overall survival.