Miloš Jeseňák, Zuzana Rennerová, Miriam Čiljaková, Zuzana Vojtušová, Ivana Plameňová, Andrea Poloučková, Peter Bánovčin
Syndromes of immune dysregulation present newly established group of inherited primary immunodeficiencies. The typical sign for these disorders is disorder of regulation of immune reactions, which are over-activated and which lead to the development of autoimmune and lymphoproliferative diseases. In the clinical picture, besides the symptoms from immune system disturbance, we can observe whole spectrum of various symptoms and signs from various organ systems. This group of immunodeficiencies includes the syndromes associated with hypopigmentation, hemophagocytic syndromes and also various autoimmune and lymphoproliferative syndromes.