[Current and future directions of pathologic analysis in hemophagocytic syndrome].
Hemophagocytic syndrome is a rare disorder with dysfunction of cytotoxic T lymphocyte (CTL) or NK cell activity, leading to excessive production of inflammatory cytokines and various clinical symptoms. HLH can be classified as either primary or secondary form; primary HLH includes familial hemophagocytic lymphohistiocytosis (FHL) and several immune deficiencies. All affected genes are involved in the transport and membrane fusion, or exocytosis of perforin/granzyme in lytic granules. Making a rapid screening of FHL with flow cytometry followed by genetic analysis is mandatory for the appropriate treatment of this fatal disease. Whereas, pathogenesis of secondary HLH is still unknown; several genetic backgrounds to affect on the pathway of T-cell activity will be associated with secondary HLH. With perforin- or Munc-deficient mouse model that develop HLH-like symptoms after virus infection, CD8+ T cells and interferon-gamma have been proven to be necessary for the HLH development. These data will provide new targets for specific therapeutic intervention of HLH in the future.
Department of Pediatrics, Ehime University Graduate School of Medicine.
Eiichi
E
Ishii
Kozo
K
Nagai
D000818:Animals; D004195:Disease Models, Animal; D020031:Epstein-Barr Virus Infections / Q000150:complications; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000209:etiology / Q000503:physiopathology; D051379:Mice