Etiology analysis of 38 patients with hemophagocytic syndrome / 中国实验血液学杂志

The study was aimed to investigate the etiology and the clinical characteristics of patients with hemophagocytic syndrome. The clinical data of 38 patients with hemophagocytic syndrome were retrospectively analyzed, and prf1 and stx11 were detected for the mutational analysis. The results showed that 38 cases were diagnosed as hemophagocytic syndrome, including 1 case of familial hemophagocytic lymphohistiocytosis (FHL), 14 cases associated with infectious disease (36.84%), 10 cases with malignancies (26.32%), 7 cases with rheumatic disease (18.42%), other 6 cases of unknown etiology (15.79%). 9 out of 38 cases died with mortality of 23.68%, including 4 cases associated with infectious disease, 2 cases with malignancies, 1 case with rheumatic disease, and 2 cases of unknown etiology. One case was found to have prf1 mutation, and was diagnosed as FHL at last. It is concluded that the causes of HPS are diverse, different etiology results in different outcome. It is important to find etiology when HPS is diagnosed, and prf1 and stx11 genetic analysis plays a important role in the diagnosis of FHL.

Transforming growth factor beta alleviates acute graft-versus-host-disease after allogeneic bone marrow transplantation in murine model / 中国实验血液学杂志

This study was purposed to investigate the effects and mechanism of transforming growth factor beta (TGF-beta) on acute graft-versus-host disease (aGVHD) after allogeneic bone marrow transplantation (allo-BMT). The recipients were male BABL/c mice, while the donors were male C57BL/6 mice. The murine model of aGVHD had been established by allo-BMT with donor derived T cells. Experiment was divided into four groups: control group, radiation control group, transplantation control group and TGF-beta treated group. Mice in TGF-beta treated group were daily subcutaneously injected TGF-beta1 (1 microg/kg) in two days before transplantation until seven days after it. The results showed that the survival time of mice in TGF-beta treated group was significantly longer than that in transplantation control group, and the aGVHD pathological changes in TGF-beta treated group were milder than that in transplantation control group. At seven days after transplantation, the level of IL-2 in TGF-beta treated group was significantly higher than that in control group, but significantly lower than that in transplantation control group. The level of IL-10 in TGF-beta treated group was significantly higher than that in transplantation control group, but the level of IL-10 in transplantation control group was significantly lower than that in other groups. It is concluded that TGF-beta may alleviate or suppress lethal aGVHD, and elevate the survival rate after allo-BMT in murine model. Accommodating of the Th1 and Th2 cytokine levels is the possible mechanism of TGF-beta preventing lethal aGVHD.

Diagnostic significance of NK cell activity and soluble CD25 level in serum from patients with secondary hemophagocytic lymphohistiocytosis / 中国实验血液学杂志

This study was aimed to explore the clinical significance of NK cell activity and serum soluble CD25 (sCD25) level in early diagnosis of the patients with secondary hemophagocytic lymphohistiocytosis (HLH). 38 suspected secondary HLH patients from June 2005 to June 2008 and 25 healthy subjects were enrolled in the study. The NK cell activity in peripheral blood was determined by a released LDH assay, The sCD25 level in serum was detected with ELISA double antibody sandwich assay. The 38 suspected secondary HLH patients were divided into diagnosed and excluded group according to HLH-2004 diagnostic criteria, The NK cell activity and sCD25 level were compared between the two groups. The results showed that 22 out of 38 suspected patients were diagnosed as secondary HLH, the NK cell activity in peripheral blood of these 22 patients was significantly lower than that of healthy control (p < 0.001), the sCD25 level in peripheral blood of these 22 diagnosed patients was higher than that of healthy control (p < 0.05). In conclusion, detection of NK cell activity and sCD25 level may be valuable in the early diagnosis of secondary HLH.

Diagnostic significance of glycosylated ferritin for patients with secondary hemophagocytic lymphohistiocytosis / 中国实验血液学杂志

This study was aimed to explore the level of glycosylated ferritin in the patients with secondary hemophagocytic lymphohistiocytosis (HLH) and its diagnostic significance. 29 suspected HLH patients from October 2007 to October 2008 were enrolled in the study, and 25 healthy subjects were selected as control. The 29 suspected HLH patients were divided into confirmed group (22 out of 29) and unconfirmed group (7 out of 29) according to HLH-2004 diagnostic criteria. The percentage of glycosylated ferritin in peripheral blood was determined by phytohemagglutinin adsorption assay. The results showed that the median level of total serum ferritin in patients of confirmed group (2897.6+/-1837.2 microg/L) was significantly higher than that in patients of unconfirmed group (653.1+/-249.1 microg/L) (p<0.01), and was also higher than that in controls (414.6+/-212.6 microg/L) (p<0.01). The median percentage of glycosylated ferritin in patients of confirmed group was significantly lower (17.0+/-4.2%) than that in patients of unconfirmed group (40.7+/-4.5%) (p<0.01) and was lower than that in controls (53.6+/-13.3%) (p<0.01). The sensitivity (86.4% vs 77.3%) and specificity (71.4% vs 42.9%) of glycosylated ferritin for the diagnosis of HLH were higher than that of total serum ferritin. In conclusions, glycosylated ferritin may be a helpful marker for the diagnosis of HLH.

Diagnostic significance of NK cell activity in the patients with hemophagocytic syndrome / 中国实验血液学杂志

This study was aimed to explore the level of NK cell activity in the patients with secondary hemophagocytic syndrome (HPS) and its significance for early diagnosis of this disease. 16 suspected HPS patients and 25 healthy subjects were enrolled in this study. The activity of NK cells in peripheral blood was detected by a released LDH assay. The activity level of NK cells in peripheral blood from patients who were finally diagnosed as HPS was compared with healthy subjects. The results showed that 8 out of 16 suspected HPS patients were finally diagnosed as secondary HPS. The activity of NK cells in peripheral blood of these 8 patients was obviously lower than that in healthy subjects with statistical significance (p<0.001), and showed abnormal in the early stage of this disease. It is concluded that the detection of NK cell activity may play an important role in earlier diagnosis of secondary hemophagocytic syndrome.