Adult hemophagocytic lymphohistiocytosis. Syndrome and disease

HLH is a specific disease and always syndrome characterized by excessive immune activation clinical analytical data of hyperinflammation that leads to short survival if the appropriate treatment delayed or not applied. It has been poorly diagnosed its incidence apparently increased in recent times, perhaps due greater recognition. Genetic /familial-based cases occur childhood from an inherited disorder poor cytotoxic function T / NK cells. In adults, majority patients have predisposing causes, such as Epstein Barr virus infection, neoplasms most commonly non-Hodgkin lymphoma origin, autoimmune processes, rarely are some classified idiopathic. Therapy based on administration LHH-94 protocol, promoted Histiocytic Society. All with genetic base require subsequent application allogeneic transplantation hematopoietic progenitors, which also therapeutic option for adults resistant, progressive recurrent.