MAS Tanı Monogenic defect in cytotoxic granule
{"Image DocumentName": "MAS Tan\u0131 Monogenic defect in cytotoxic granule", "Image ImageDescription": "exocytosis (FHL genleri) VEYA. HLH-2004 kriterleri \u22655/8. Klinik: Ates, Splenomegaly. Lab:, Bisitopeni:Hb<9, Neutrophils<1000, Platelets<100. Ferritin>500mg/l. Fibrinogen<150, Triglycerid>265mg/dl. sCD25 ( soluble IL2 receptor)>2400U/ml, NK hucre akt\u0131v\u0131tes\u0131nde azalma. Hemofagositozun gosterilmesi. Mild MAS. IL6, IL4, IL1, IL18, IL33, cytotoxic granule function. IL10. healthy. Severe MAS. Pathway of cytotoxic T cell killing or NK cell killing; missing perforin or genes to move perforin to membrane to secrete it. Gene expression profiling with high ferritin. If Il18 group, tend to get MAS; Il6 more vs. il18 vs; transcriptional profile that will result in MAS. Innate Immune driven MAS: Drive cytokines by stimulating TLR9 (pathogen DNA sensor); IL12 leads to IFN\u03b3. If block IL10, can cause hemophagocytosis. Hyper IL4 leads to MAS. IFN\u03b3. Behrens, ACR 2014.", "Image XResolution": "37", "Image YResolution": "37", "Image ResolutionUnit": "Pixels/Centimeter", "Image Software": "SlidePlayer", "Image YCbCrPositioning": "Centered"}