Publication: Inherited Hemophagocytic Lymphohistiocytosis (HLH)
Inherited Hemophagocytic Lymphohistiocytosis (HLH)
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Pachlopnik Schmid, J., & de Saint Basile, G. (2010). Inherited Hemophagocytic Lymphohistiocytosis (HLH). Klinische Pädiatrie, 222, 345–350. https://doi.org/10.1055/s-0029-1246165
Abstract
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Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal immune disorder characterized by uncontrolled lymphocyte- and macrophage-activation. The resulting hypercytokinemia and cell infiltration of organs lead to the clinical and laboratory features of HLH. Viral infections and other triggers can induce both, inherited and acquired forms of HLH. Disease-causing mutations in the genes encoding perforin (PRF1, FHL2), munc13-4 (UNC13D, FHL3), syntaxin 11 (STX11, FHL4), and munc18-2 (UNC18-2/STXBP2, FHL5) have been previously ident
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- de Saint Basile, G
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Pachlopnik Schmid, J., & de Saint Basile, G. (2010). Inherited Hemophagocytic Lymphohistiocytosis (HLH). Klinische Pädiatrie, 222, 345–350. https://doi.org/10.1055/s-0029-1246165
