| Objective In order to analyze the clinical features,etiology and prognosis of hemophagocytic lymphohistiocytosis(HLH).Methods The clinical data of 125 patients with HLH from June 2010 to August 2017 in Fujian Medical University Union Hospital were retrospectively analyzed.125 patients were divided into malignancy-associated hemophagocytic lymphohistiocytosis(M-HLH)and non-malignancy-associated hemophagocytic lymphohistiocytosis(non-M-HLH)group.Clinical features,laboratory parameters and survival of the two groups were compared in totality,pediatric and adult patients respectively.Results(1)Among the 125 patients,there were 39 with M-HLH,including 28 males,11 females,and 86 with non-M-HLH,including 42 males and 44 females.Ages of M-HLH patients ranged from 2 to 68 years old(median age : 19).nonM-HLH patients were 0.25 to 68 years old(median age: 4).(2)The 39 patients with M-HLH consisted of 33 cases of lymphoma,4 cases of leukemia and 2 cases of myelodysplastic syndrome.The 86 patients of non-M-HLH included 80 infection related cases and 6 rheumatic immune related cases.(3)Among all patients,gender,age,alanine aminotransferase,aspartate aminotransferase,direct bilirubin,indirect bilirubin,albumin,serum creatinine,NK cell ratio were statistically significant difference between the M-HLH and non-M-HLH group(p < 0.05).Multivariate Logistic regression analysis identified aspertate aminotransferase,albumin,direct bilirubin as independent factors which were associated with M-HLH.In children,M-HLH and non-M-HLH patients were statistically significant different in age,alanine aminotransferase,aspertate aminotransferase,direct bilirubin,indirect bilirubin,NK cell ratio(p < 0.05).Multivariate Logistic regression analysis not showed statistical correlation between those factors and M-HLH.In the adult group,aspertate aminotransferase,directbilirubin,indirect bilirubin,CD4+ cell ratio,NK cell ratio had difference in M-HLH and non-M-HLH patients(p < 0.05).Multivariate Logistic regression analysis not showed statistical correlation between those factors and M-HLH.ROC curve analysis showed that the optimal predictive value of NK cells ratio in totality and pediatric group was 4.96% for diagnosing M-HLH.The optimal predictive value of NK cells ratio in the adult group for diagnosing M-HLH was 2.08%.(4)Followed up for 93.8 months,the median overall survival(OS)was 50.4 months in all patients.Median OS of non-M-HLH patients was 68.1 months,which were significantly better than 15.3 months in M-HLH patients(p < 0.05).Single factor analysis results showed that age,platelets,alanine aminotransferase,aspertate aminotransferase,albumin had an effect on survival(p< 0.05).COX regression analysis results showed that platelets and albumin were independent factors affecting survival.The median OS of the pediatric HLH group was 76.4 months.In pediatric,non-M-HLH patients showed superior OS than M-HLH patients(78.9m vs 32.2m,p= 0.011).Single factor analysis showed that alanine aminotransferase,aspertate aminotransferase,albumin and platelet,direct bilirubin,indirect bilirubin had an impact on survival(p< 0.05).While COX regression results showed that those above factors didn't affect survival.The median OS of adult patients was 20.7 months.Compared with 44.3 months' s median OS in adult non-M-HLH patients,adult M-HLH patients suffered inferior prognosis,with median OS of 7.73 months(p= 0.011).Platelets and albumin level had an effect on survival,using single factor analysis and verified as independent survival factors by COX regression test.Conclusion The OS of non-M-HLH was significantly better than M-HLH in totality,pediatric and adult patients respectively.The proportion of NK cells ratio has predictive value for the diagnosis of M-HLH.Among all patients,non-M-HLH was more prone to liver dysfunction than M-HLH.The platelet count and serum albumin level were related to the worse prognosis.Moreover,the correlated of platelet count and serum albumin level worse prognosis were as well as in adult HLH patients. |
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