| Research Background Hemophagocytic syndrome(hemophagocytic syndrome, HPS) also known as hemophagocytic lymphohistiocytosis hyperplasia(Hemophagocytic Lymphohistiocytosis, HLH) is divided into primary or hereditary and secondary categories. Many patients with primary or hereditary blood family history or genetic mutations proved to have clear, so there is high probablity to catch this in infant and childhood stage; While for secondary infection, cancer, autoimmune, medicine and transplantation are often the main cause, it is more common in adults. Low morbidity but high mortality rates, the clinical symptoms include a persistent high fever, hepatosplenomegaly, convulsions and nervous system damage may occur in patients with severe, atypical because early symptoms, diagnosis is very difficult, and progress quickly, many patients unable to clear for early diagnosis, not treated lead to death. Early diagnosis is essential for reducing mortality in patients, because there is no recognized diagnostic criteria and the lack of verifiability of the gold standard, as soon as possible a clear diagnosis continues to be a serious challenge for clinicians. With constantly developing on the disease awareness, in recent years cases reported patient gradually increased, but disease mechanism is not clear, currently bite blood cell integrated syndrome specific onset mechanism unknown, but most think immune system participated into the processing of this disease, that: bite blood cell integrated syndrome exists promoting apoptosis died signal and inherent immune and the adaptability immune reaction related gene expression cut, and collaborative coding promoting inflammatory factor and anti-apoptosis died factor of gene expression raised of phenomenon. The views think, gene defects,and infection and the tumor, causes caused body immune system disorder, makes t cell continued proliferation, Th1/Th2 proportion imbalance, Th1 excessive activation, produced large of IFN-, activation CD8+ cell generated large cell factor, led to giant bite cell large activation, activation of giant bite cell and secretion large of inflammatory sex factor, as: IL-12, and IL-10, and IL-18, and TNF-alpha and the other IL pigment class, Which IL-12 can induced secretion Thl cell factor of cell development and promote these cell and NK cell produced IFN-, formed cycle, this is so-called of "cell factor storm" theory, this series not by inhibit of effect makes CTLs activated and Th cell migration, caused end late organ not reversible sex injury; and due to bite blood cell integrated syndrome in the lymph nodes enlargement, and blood cell reduced and gel blood function obstacles, symptoms are by "cell factor storm" Systemic reactions to inflammation mainly caused by lead, test helps diagnose on inflammatory factors in patients with hemophagocytic syndrome and prognosis. This group studied through analysis of inflammatory cytokines levels in patients with HPS, we intend to look for HPS indicators gap between patients and healthy people, and the different stages in a group of patients with HPS, population with different outcomes were observed, to help us understand the early diagnosis of HPS and the course of judgment.Research purposes His research through detection bite blood cell integrated syndrome and normal health crowd peripheral blood serum IL-8, TNF-alpha, IL-6, and IL-10 of level changes,compared two group between inflammation factor expression level differences, discussion the function of serum IL-8, and IL-10, and IL-6, and TNF-alpha during clinical diagnosis, and the correlation of HLH during the clinical performance, in additional, the before and after of changes trend in HLH treatment, then analyze its’ value for application on disease judge and prognosis assessment.Research methods We selected 28 patients with hemophagocytic syndrome, who have been diagnosised and treatment in the first affiliated hospital of Zhengzhou University Department of Hematology, Apply enzyme joint immune adsorption method(ELISA) detection them and other the 20 cases health people’s serum IL-8, and IL-10, and IL-6, and TNF-alpha of level changes, then compared two group inflammation factor expression level differences; and divided all patients into acute attack period and recovery period, respectively has 16 people and 12 people, compare different patients above several items index serum level; and follow-up a year, observe bite blood cell integrated syndrome patients death cases within follow-up period, compared death and survived patients between IL-6, and IL-8, IL-10, TNF-α level differences. Operate strictly according tothe equipment and Kit instruction; inflammatory cytokines levels of measurement data(x ± s) that used t test, P<0.05 has significant differences.Result 1.Comparison of two group expression of inflammatory factor: We found patients with hemophagocytic syndrome the level of blood serum in IL-6(36.7±8.4pg/ml) 、 IL-8(56.9±10.1pg/ml) 、 IL-10(194.3±23.9pg/ml) 、 TNF-α(16.5±2.0pg/ml) was significantly higher than the index of healthy reference level(4.5±0.8pg/ml、5.7±1.2pg/ml、3.0±0.5pg/ml、2.8±0.7pg/ml)(t=51.016,t=66.871,t=108.192,t=110.887,P<0.01)。 2. Comparison of expression of inflammatory factors: Between acute and recovery patients with acute patients IL-6(44.8±11.3pg/ml)、IL-10(240.7±32.5pg/ml)level was significantly higher than patients in remission period(24.8±7.5pg/ml、126.0±26.2pg/ml)(t=14.390,t=26.337,P<0.01)。 3.Difference between different prognosis expression of inflammatory factors: The death Group IL-6(121.0±15.6pg/ml)和 TNF-α(22.7±6.3pg/ml)expression was significantly higher than the survival Group(16.1±4.6pg/ml、15.0±3.7pg/ml)(t=39.923,t=7.042,P<0.01)。Conclusion 1, Patients with hemophagocytic syndrome IL-6, and IL-8, and IL-10, and TNF-alpha level significantly higher than health control group,So we speculated that these factors in hemophagocytic syndrome, inflammation plays an important role. These joint indicators were significantly higher for early diagnosis of hemophagocytic syndrome overlaps meaning; 2,Acute period patients IL-6, and IL-10 level significantly above ease period, but IL-8 and TNF-alpha level differences no statistics meaning,so that have some indicative effectiveness to detect the disease, and severity of the illnesses, but for the judgment of acute or sustained there was little significance. 3,The Group of death patients IL-6 and TNF-alpha expression level was significantly higher than the survival group, confirmed that cytokines in patients with IL-6 and TNF-alpha levels and the severity of the disease, that is associated by inflammatory destruction of the body. 4, So combined detection of above index have some meaning to screen symptom. |
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