Familial hemophagocytic lymphohistiocytosis (FHL) is a group of progressive and potentially lethal inflammatory diseases caused by a deficiency in proteins involved in cytolytic granule formation or release.
La linfohistiocitosis hemofagocítica familiar (FHL) es un grupo de enfermedades inflamatorias progresivas y potencialmente letales causadas por deficiencia de proteínas involucradas en la formación o liberación de gránulos citolíticos.
| Front | A family of progressive and potentially lethal inflammatory diseases caused by an inherited deficiency of one of several proteins involved in the formation or release of cytolytic granules. Large numbers of polyclonal CD8-positive Tregcells accumulate in lymphoid and other organs, and this is associated with activated macrophages that phagocytose blood cells, including erythrocytes and leukocytes |
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| Back | familial hemophagocytic lymphohistiocytosis FHL |
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