| Objective:To investigate clinical features, effective treatment programs and prognosis related factors of hemophagocytic syndrome.Methods:We retrospectively analyzed clinical information of 62 patients diagnosed with hemophagocytic syndrome from August 2006 to October 2014 in the Second Affiliated Hospital of Fujian Medical University, including age, gender, etiology, clinical presentation, laboratory parameters and different treatment options, used SPSS17.0 software for the statistical data, used Cox regression model with single factor and multiple factors analysis, used product-limited method(Kaplan Meier) to draw the survival curves and the Log- Rank test, the P value <0.05 was considered statistically significant.Result:(1) male/female: 1.14 :1, the median onset age was 50 years old, range from 16 to 82 years old.(2) Proportion of unknown cause was 38.7%; infection was 29%, including EB virus 8.1%; secondary tumor was 24.2%, 21% of which lymphoma; immune-related by 8.1%. The survival curves of different causes showed secondary tumor survival rate was lowest, with the median survival time of 11 days, one month survival rate of 14.4%, half a year to zero.(3) Clinical features mainly had fever(98.4%), liver enlarge(33.9%), spleen enlarge(67.7%), lymph node enlargement(46.8%), respiratory symptoms(59.7%), neuropsychiatric symptoms(25.8%), serous cavity effusion(62.9%) and so on.(4) Laboratory characteristics mostly included peripheral blood cells decrease(83.9%), bone marrow hemophagocytic phenomenon(78%), high triglycerides(66.1%), lowfibrinogen(51.6%), high ferritin(97.9%), high lactate dehydrogenase enzyme(88.7%), high alanine aminotransferase(74.2%), high blood urea nitrogen(27.4%) and so on.(5)The different treatment options’ survival curves showed the survival rate of etiological treatment combined hormone and gamma globulin was higher, the median survival time was 527 days, and the survival rates at 0.5 year, 1 year, 3 years were 75%, 54.7%, 36.5% respectively.(6)The COX model multivariate analysis showed age(P=0.004), neurological symptoms(P=0.005), bone marrow hemophagocytic phenomenon(P=0.002) and treatment programs(P=0.001) were statistically significant.Conclusions:(1)HPS etiology was diversity, which infections were the most common in the clear cause; followed by tumor, which the majority were lymphoma patients and poor prognosis; higher percentage of unknown cause, which might still contain some uncertain pathogeny biology.(2) Complex and varied clinical manifestations of HPS patients and the lack of specificity, so to alert neuropsychiatric symptoms, and it prompted the disease might progress to terminal and the prognosis was poor.(3) Hemophagocytic phenomenon was not a specific index for the diagnosis of HPS, but when hemophagocytic phenomenon occurred such as bone marrow, we should be highly suspected HPS, and it might indicate a poor prognosis, timely intervention.(4) For the high degree of suspicion in patients with HPS in the clinical, they shoud be early diagnosis and timely intervention, especially for patients with infection related or unclear reasons, as soon as possible to the anti-infective drugs, and to the hormone and gamma globulin therapy, which could improve the prognosis. For lymphoma-associated HPS, the prognosis was very poor and needed to further explore the positive and effective interventions. |
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