Article
Advances in the pathogenesis of primary and secondary haemophagocytic lymphohistiocytosis: differences and similarities.
British journal of haematology - 1 Jul 2016
Brisse Ellen, Wouters Carine H, Matthys Patrick
Abstract excerpt
Haemophagocytic lymphohistiocytosis (HLH) comprises a heterogeneous spectrum of hyperinflammatory conditions that are inherited (primary HLH) or acquired in a context of infections, malignancies or autoimmune/autoinflammatory disorders (secondary HLH). Genetic defects in the cytotoxic machinery of natural killer and CD8(+) T cells underlie primary HLH, with residual cytotoxicity determining disease severity....
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