| Objective The aim of this study isto retrospectively analyze the clinical baseline data,treatment efficacy and prognosis of adult secondary hemophagoctic lymohohistocytosis(s HLH)associated with different primary diseases,and to evaluate the efficacy of different treatment regimens.The factors affecting the outcome were explored.In order to improve the understanding of this kind of disease,so as to achieve early diagnosis and treatment.Methods The clinical data of 122 adult patients with s HLH who were initially diagnosed and treated in the General Hospital of Ningxia Medical University from January2015 to December 2021 were collected,and 81 patients who met the diagnostic criteria were screened out:(1)According to the etiology,the patients were divided into three groups:infection-related group,malignant tumor-related group and autoimmune-related group.The age,gender,diagnosis time,etiology,clinical manifestations and laboratory indicators of the patients were compared and analyzed.(2)The response and outcome of patients in infection-related group,malignant tumor-related group and autoimmune-related group were analyzed.Results1.1.Among 81 cases of adult s HLH,42 were male and 39 were female,male: female=1.08:1.The onset age ranged from 20 to 78 years old,and the median age was 52 years old.2.Among the 81 patients,49 cases(49/81,61%)were related to infection,among which12 cases were infected by EB virus at most.There were 23 cases(23/81,28%)associated with malignant tumor,among which NK/T cell lymphoma accounted for 12 cases at most.Autoimmunity was related in 9 cases(9/81,11%),among which 5 cases were systemic lupus erythematosus.3.Clinical symptoms and signs: 76 patients(76/81,93.8%)developed persistent high fever,16 patients(16/81,19.6%)developed rash,6 patients(6/81,7.4%)developed central diseases,and 9 patients(9/81,11.1%)developed hepatomegaly.The incidence of rash in autoimmune related group was higher than that in infection related group and malignant tumor related group(6/9,66.67%),and the incidence of lymphadenopathy in malignant tumor related group was higher(17/23,73.91%).The results showed that there was a statistical difference in the number of patients among the three groups(P<0.05).In terms of laboratory examination,the autoimmune related group had statistical differences in the levels of total bilirubin,total cholesterol,prothrombin time and activated partial thromboplastin time(P <0.05).The level of direct bilirubin in infection-related group was significantly lower than that in other two groups(P<0.05).4.All 81 patients received relevant treatment,of which 34 patients got better and were discharged.17 patients whose symptoms were not relieved were transferred to higher hospitals for further treatment;25 patients gave up treatment;Five patients died in hospital without control.The results showed that the treatment scheme had no effect on the short-term prognosis(P=0.094).Up to the end of the follow-up,23 patients only received general supportive treatment,24 patients received glucocorticoid plus general supportive treatment,23 patients received HLH-94/HLH-2004 regimen(etoposide+cyclosporine)and 11 patients received other chemotherapy regimens.The median survival time and 1-year,3-year and5-year survival rates of HLH-94/HLH-2004 regimen are higher than those of other treatments,but the treatment regimen has no effect on the long-term prognosis of adult s HLH(P=0.235).5.By the end of the follow-up,the overall median survival time was 1.19 months,and the median survival time of the patients in the infection-related group was 1.5 months.The1-year,3-year and 5-year survival rates were 44.2%,36% and 36% respectively,among which the median survival time of patients infected with EB virus was 0.46 months,and the1-year,3-year and 5-year survival rates were 30.0%,10.0% and 10.0% respectively.The median survival time of patients with malignant tumor was 0.46 months,and the 1-year,3-year and 5-year survival rates were 13.6%,9.1% and 0% respectively,among which the median survival time of patients with lymphoma was 0.4 months,and the 1-year,3-year and5-year survival rates were 15.0%,10.0% and 0% respectively.The 1-year,3-year and 5-year survival rates of autoimmune patients were 88.9%.Survival analysis showed that the survival rate of autoimmune patients was higher than that of infection-related group and malignant tumor-related group(P<0.001).6.Univariate analysis showed that the absolute value of neutrophils,monocytes,alanine aminotransferase,aspartate transferase,total protein,total bilirubin,total cholesterol,direct bilirubin,white blood cell count,activated partial thromboplastin time,prothrombin time,fibrinogen,serum ferritin and rash had significant differences in the prognosis of patients(P<0.05).COX multivariate analysis showed that rash was an independent related factor affecting survival.Conclusion1.The etiology of adult s HLH is complicated,and infection and tumor are the most common.Among them,EB virus infection and lymphoma are the most common.Fever,splenomegaly and lymphadenopathy are the most common symptoms,among which rash is an independent related factor for the prognosis of adult s HLH patients,but it still needs to be confirmed by expanding the number of samples and multi-center research.Laboratory examination showed that the total bilirubin level decreased,the prothrombin time was short,and the time to activate partial thromboplastin was shortened,which was of diagnostic significance in autoimmune-related s HLH,while the direct bilirubin level increased in infection-related s HLH.2.Adult s HLH patients who receive treatment,although the treatment scheme is different,have no effect on the short-term and long-term prognosis of patients.3.According to the outcome of the etiological patients,among the infection-related adult s HLH,patients infected with EB virus have a worse prognosis than other infections.In adult s HLH associated with malignant tumors,the prognosis of lymphoma patients is worse than that of other malignant tumors.The overall prognosis of autoimmune-related adult s HLH is good.The final outcome showed that lymphoma patients had the worst prognosis. |
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