Mezenşimal Kök Hücrelerin İmmün Modülatör Etkisinin Hemofagositik Sendrom Klinik Uygulamalarına Temel Oluşturmak Üzere İn Vitro Modelde Incelenmesi

Abstract

If untreated, Hemophagocytic syndrome (Hemophagocytic Lymphohistiocytosis, HLH), is a fatal immunodeficiency disease, which results from the over activation of cytotoxic T lymphocytes and macrophages caused over activated immune system. A type of primary hemophagocytic syndrome FHL2 is a perforin gene is mutated. Perforin is the key protein for immune response of cytotoxic T cells and natural killer (NK) cells. Cytotoxic T lymphocytes and NK cells secrete their perforin and granzyme containing granules when they contact virus-infected cells or tumor cells. Perforin creates pores at the target cell membrane, and mediates passing granzyme through these pores and inducing apoptotic death of the target cell. Because of the perforin gene mutation in FHL2, these patients do not regulate their immune systems and continually stimulated immune response in these patients causes high levels of cytokine secretion.