Analysis Of Clinical Features And Discussion Of Different Treatment Regimens In Lymphoma-Associated Hemophagocytic Syndrome

PART1 CLINICAL SIGNATURES OF LYMPHOMA-ASSOCIATED HEMOPHAGOCYTIC SYMDROMEObjectiveIn this study,clinical indicators such as the expression level of Th1 /Th2 cytokines and the distribution of lymphocyte subsets were analyzed to assist in the diagnosis of lymphoma-associated hemophagocytic syndrome.MethodsA total of 131 patients with lymphoma-associated hemophagocytic syndrome,158 patients with lymphoma without hemophagocytic syndrome and 27 normal healthy volunteers were collected to study.The differences in the expression levels of Th1 / Th2 cytokines [IL-2,IL-4,IL-6,IL-10,tumor necrosis factor-α(TNF-α)and γ-interferon(IFN-γ)] and lymphocyte subsets(CD3+,CD4+,CD8+,CD19+,CD4+/CD8+,and CD16+/56+)in three groups of patients were detected and analyzed retrospectively.Results1.Comparison of Th1 / Th2 cytokine expression levels: Pair comparison showed that the levels of IL-4,IL-10 and IFN-γ in patients with lymphoma-associated hemophagocytic syndrome were significantly higher than those in patients without lymphoma-associated hemophagocytic syndrome and normal healthy volunteers(all P <0.05).The expression of IL-6 in patients with lymphoma-associated hemophagocytic syndrome was significantly higher than that in healthy volunteers(P < 0.05),but there was no significant difference between the expression of IL-6 in patients with lymphoma-associated hemophagocytic syndrome and those without lymphoma-associated hemophagocytic syndrome(P > 0.05).2.Comparison of lymphocyte subsets distribution: Pair comparison showed that CD19+ distribution in patients with lymphoma-associated hemophagocytic syndrome was significantly lower than that in normal healthy volunteers(P < 0.05),but there was no significant difference in CD19+ distribution between patients with lymphoma-associated hemophagocytic syndrome and patients without lymphoma-associated hemophagocytic syndrome(P > 0.05).Conclusion The expression levels of IL-4,IL-10 and IFN-γ in patients with lymphoma-associated hemophagocytic syndrome were significantly higher than those in patients without lymphoma-associated hemophagocytic syndrome and normal healthy volunteers,and monitoring their expression levels is helpful for the diagnosis of lymphoma-associated hemophagocytic syndrome.PART2 THE STUDY OF RUXOLITINIB COMBINED WITH DOXORUBICIN,ETOPOSIDE,AND DEXAMETHASONE AND HLH-94 FOR THE TREATMENT OF THE LYMPHOMA-ASSOCIATED HEMOPHAGOCYTIC SYNDROMEObjectiveThe clinical characteristics,laboratory indicators,survival analysis and gene sequencing analysis of patients with lymphoma-related hemophagocytic syndrome were analyzed and compared after different treatment regimens,and the treatment methods of lymphoma-related hemophagocytic syndrome were discussed,so as to provide reference for anti-hemophagocytic treatment of patients with lymphoma-related hemophagocytic syndrome.MethodsThe clinical characteristics,infection indexes,liver function,lactate dehydrogenase,serum ferritin,Th1 / Th2 cytokines,lymphocyte subsets and other laboratory test indexes of 81 patients with lymphoma-associated hemophagocytic syndrome admitted to the Department of Hematology,the First Affiliated Hospital of Guangdong Pharmaceutical University from June 2018 to December 2020 were retrospectively analyzed.The differences of different treatment regimens were compared in terms of clinical test indexes,clinical characteristics,treatment response and effect,and final survival.Results1.81 cases of lymphoma-associated hemophagocytic syndrome By lymphoma pathology,48 cases(59%)were NK/ T-cell lymphoma,13cases(16%)were diffuse large B-cell lymphoma,15 cases(19%)were peripheral T-cell lymphoma,and 5 cases(6%)were other lymphoma.2.The Clinical features of the patients were as follows: fever(85.2%),spleen enlargement(58%),complete blood reduction(81.4%),high triglycerides(>3mmol/L,51.2%),low fibrinogen concentration(< 1.5g/L,46.9%),hemophagocytosis in bone marrow(84%),high serum ferritin(> 500 ng/ml,95%),reduction or absent of NK cell activity is reduced(100%),increased s CD25(soluble interleukin-2 receptor)(≥2400U/ml,94.3%),positive plasma EBV-DNA(86.4%),liver function impairment(68%),increased IL-10(92.6%),increased IL-6(88.9%),increased IFN-γ(52.5%),decreased CD4+ T cells(46.7%),CD4+/CD8+<1(46.9%),decreased NK cell population(35.8%),increased lactate dehydrogenase(87.7%),etc.3.Two groups of patients in the diagnosis of the type of lymphoma that eats blood distribution period of Ann Ar Bor,lymphoma,refractory lymphoma/recurrence ratio,ECOG score,fever,blood cells,blood triglycerides,reduce or iron protein levels,liver function abnormalities,fibrinogen,IL-6,IL-10,the gamma interferons,CD4 +,CD4 + / CD8 + T cells and NK cells,EBV DNA level had no statistical difference(P >0.05).4.Rank sum test showed that the body temperature,white blood cell count,IL-4,IL-6,IL-10,IFN-γ,TNF-α,C-reactive protein,procalcitonin,lactate dehydrogenase and other values were decreased in the R+DEP treatment group at about 5 days after treatment.The difference was statistically significant(all P<0.05).Other related indexes,such as neutrophils count,IL-2,serum ferritin and Epstein-Barr virus,all decreased at 10-15 days(all P < 0.05),but the distribution of lymphocyte subsets did not change significantly before and after treatment.5.The Shrinkage rate of spleen in the R+DEP group was significantly better than that in the HLH-94 group.The difference was statistically significant(P<0.05).6.The response rate in the R+DEP group was higher than that of HLH-94 group.The difference was statistically significant.(P<0.05).The mortality rate of R+DEP group was significantly lower than that of HLH-94 group.The difference was statistically significant(P<0.05).7.The OS was signifcantly prolonged in the R+DEP group compared with the HLH-94 group.The difference was statistically significant(P<0.05).Conclusion In this study,compared with the HLH-94 regimen,the R+DEP regimen showed more obvious curative effect,the total effective rate was significantly higher than that of the HLH-94 regimen,and the mortality rate was significantly lower than that of the HLH-94 regimen.The early use of R+DEP regimen in LAHS patients can control the cytokine storm in time,reduce the mortality rate and improve the overall survival rate.