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- W2494535509 endingPage "460" @default.
- W2494535509 startingPage "437" @default.
- W2494535509 abstract "Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of severe hyperinflammation caused by uncontrolled proliferation and activation of lymphocytes and macrophages secreting high amounts of inflammatory cytokines. Clinical hallmarks are prolonged fever, hepatosplenomegaly, neurological symptoms, and cytopenias. Genetic defects in familial forms of HLH (FHL), and in some immune disorders associated with partial albinism (Chediak-Higashi, Griscelli, and Hermansky-Pudlak syndromes), impair the function of natural killer and cytotoxic T lymphocytes. This leads to defective killing of target cells and a failure to control the immune response. Despite progress in diagnostics and therapy, mortality of patients with severe HLH remains high. Reference treatment is an etoposide-based protocol to induce remission, but new approaches are currently being explored. In inherited HLH, hematopoietic stem cell transplantation is the only curative option. Current research aims at a better understanding of disease pathogenesis and evaluation of more targeted therapeutic approaches, including anti-cytokine antibodies and gene therapy." @default.
- W2494535509 created "2016-08-23" @default.
- W2494535509 creator A5038630174 @default.
- W2494535509 creator A5047506862 @default.
- W2494535509 date "2014-01-01" @default.
- W2494535509 modified "2023-10-03" @default.
- W2494535509 title "Genetic Diseases Predisposing to HLH" @default.
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