Adult hemophagocytic lymphohistiocytosis. Syndrome and disease

Abstract

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