Secondary haemophagocytic lymphohistiocytosis associated with infectious mononucleosis

Hemophagocytic lymphohistiocytosis (HLH) is a progressive multisystem heterogeneous disease arising on the basis of a specific congenital (primary HLH) or acquired (secondary HLH) disorder of the immune system. It is a syndrome characterized by uncontrolled proliferation of activated T-lymphocytes, NK (natural killer) cells and macrophages. Excessive secretion of cytokines and hemophagocytic activity of macrophages in the lympho-reticular system and central nervous system (CNS) results in the development of typical clinical and laboratory signs. (author)