Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China.

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality. METHODS: To improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014. RESULTS: Patients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT) <40 × 10(9)/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 10(9)/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019). CONCLUSIONS: HLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 10(9)/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.

Identification of clinical features of lymphoma-associated hemophagocytic syndrome (LAHS): an analysis of 69 patients with hemophagocytic syndrome from a single-center in central region of China.

To identify the clinical features of lymphoma-associated hemophagocytic syndrome (LAHS), we retrospectively analyzed the clinical characteristics, laboratory findings and survival data of 16 LAHS patients from 69 adult hemophagocytic syndrome (HPS) patients. The results showed that the most common clinical manifestations and laboratory parameters were fever (100%), ferritin ≥ 500 g/L (100%), peripheral cytopenia in two or more lineages (100%), fibrinogen (Fbg) < 1.5 g/L (93.8%) and splenomegaly (81.3%) in LAHS patients. The percentages of patients with Fbg < 1.5 g/L, PLT < 40 × 10(9)/L and LDH ≥ 1,000 U/L in the LAHS group were significantly higher than those in non-LAHS patients (P = 0.010, 0.000, and 0.001, respectively). Survival analysis showed that HLH patients with rheumatological reasons had better prognosis (OS; median not reached), followed by patients in the infection group (350 days) and those with unexplained causes (140 days). LAHS had the worst prognosis (only 37 days). The symptoms of LAHS patients are usually confused with other HPS. Patients with LAHS had higher probabilities to have Fbg < 1.5 g/L, PLT < 40 × 10(9)/L, LDH ≥ 1,000 U/L and poor prognosis, so early diagnosis and systemic treatments are required.

[Meta-analysis of intervention effects on obesity in Chinese pupils].

OBJECTIVE: To comprehensively evaluate the effect of interventions on obesity in Chinese pupils. METHODS: A literature research was carried out in China National Knowledge Infrastructure, Wanfang Data, VIP Database for Chinese Technical Periodicals, PubMed and the Excerpts Medica Database (EMBASE) databases to collect articles published between 1979 and 2010 concerning the effect of interventions for preventing obesity in Chinese pupils. Rate difference (RD) of the rate of obesity as the evaluation indicator was selected to Meta-analyze the effect of interventions on obesity. There are total 215 articles, in which 211 articles were written in Chinese and other articles were written in English. RESULTS: 17 literatures were used for Meta-analysis by the uniform inclusion and exclusion criteria. The results showed that the RD of obesity rate for the students in the intervention group was 3% (95%CI: 1% - 5%) after the intervention. However, the RD of obesity rate for the students in the control group was -2% (95%CI: -4% - -1%) after the intervention. Results of stratified analysis for the RD of obesity rate showed that the obesity rate for the students in the intervention group were decreased significantly after the intervention of combined programs with health education, physical exercise and nutrition interventions with moderate intervention time (1 to 2 years), the RD (95%CI) of obesity rate were 5% (2% - 8%), 3% (1% - 4%), respectively. For the studies whose baseline obesity rates was insignificant difference between the intervention group and the control group, the obesity rate for the students in the intervention group was 4% (95%CI: -7% - -1%) lower than the obesity rate for the students in the control group after the intervention. CONCLUSION: Health-education-based comprehensive intervention is effective on obesity prevention in Chinese pupils; combined intervention programs with moderate intervention time (1 to 2 years) were effective in improving efficiency of obesity prevention in pupils.

[Meta-analysis on the relationship between leptin receptor Gln223Arg and Pro1019Pro gene polymorphism and obesity in the Chinese population].

OBJECTIVE: To evaluate the associations between polymorphisms of LEPR Gln223Arg, LEPR Pro1019Pro and the risk on obesity. METHODS: A computerized search on literature was carried out in Wanfang, CNKI, VIP databases and CBM, PubMed, EMBASE databases to collect articles published between 1979 and 2010 concerning the associations between polymorphisms of LEPR Gln223Arg and/or LEPR Pro1019Pro and risk of obesity in the Chinese population. Odds ratios (ORs) were used to assess the strength of the association, and 95% confidence intervals (CIs) to present the precision of the estimates. Meta-analysis was performed using the STATA statistical software. RESULTS: Fifteen literature were collected for Meta-analysis by the uniform inclusion and exclusion criteria. There were 1096 obese patients and 949 controls for polymorphisms of LEPR Gln223Arg in 9 papers, together with 961 obese patients and 818 controls for polymorphisms of LEPR Pro1019Pro in 8 papers. Overall, there were significant associations between decreased risk of obesity and LEPR Gln223Arg polymorphisms (-668 A→G) (G versus A, OR = 0.66, 95%CI: 0.49 - 0.89; AG and GG versus AA, OR = 0.50, 95%CI: 0.32 - 0.77; respectively). There were significant associations between increased risk of obesity and LEPR Pro1019Pro polymorphisms (-3057 G→A) (A versus G, OR = 1.61, 95%CI: 1.15 - 2.26; AG and AA versus GG, OR = 1.50, 95%CI: 1.08 - 2.08; respectively). CONCLUSION: Variant alleles at both LEPR-668 and LEPR-3057 were associated with obesity in the Chinese Han-dominated population.