Blood: Cytotoxic gene mutation sychagic syndrome for secondary phagocytosis

Phagocytophaphal syndrome (HLH) is a life-threatening high-inflammatory disease, mainly due to cytotoxic killer cells (CTL) and NK cell functional defects leading to antigen removal disorders, mononucleic macrophage system received continuous antigen stimulation and over-activation proliferation, resulting in a large number of inflammatory cytokines caused by a group of clinical syndromesThe main manifestations are fever, large spleen, reduction of whole blood cells, high triglycerides, low fibrinogen, high serum ferritin, and can be found in bone marrow, spleen or lymph node biopsyprimary HLH is generally older due to the cytotoxicity of lymphocytes due to single-gene binary allele mutations, most of which occur within 1 year of ageSecondary HLH occurs more frequently in adults, secondary infections, lymphoma or rheumatismthe cytotoxic status of lymphocytes insecondary HLH has not been clarifiedThe researchers now conducta a systematic assessment of NK cytocellular toxicity in adult patients with secondary HLHcompare total lymphocyte count and subtype, NK cell phenotype, perforated expression and granulation, natural or ADCC cytotoxicity in adult patients with secondary HLH( case control, DC) and healthy people (healthy control, HC)researchers systematically screened cytotoxic gene mutationsThe HLH group included 68 patients and the DC group and the HC group were included in 34-bit control, respectivelyin HLH patients, severe and transient lymphocyte reduction, activated NK cell phenotypes (CD69, ICAM-1, HLADR, CCR5 expression increased) and interferon radon productionThe average expression of perforated hormone was normal, and there was no difference between departicle test and NK cytotoxicity and THE DC groupSingle allele or perforated A91V mutation serotonin in nearly 50% of patients were observedcompared to DC, the researchers did not find major internal cytotoxic dysfunction or predicted pathogenic gene mutations in secondary HLHThe active NK phenotype, associated with the reduction in interferon radon production, is similar to other highly inflammatory diseases such as sepsis or systemic juvenile ispot arthritis