Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy.

@article{Gupta2010PrimaryAS,
  title={Primary and secondary hemophagocytic lymphohistiocytosis: clinical features, pathogenesis and therapy.},
  author={Sumit Gupta and Sheila Weitzman},
  journal={Expert review of clinical immunology},
  year={2010},
  volume={6 1},
  pages={137-54}
}
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal hyperinflammatory syndrome with prolonged high fever, hepatosplenomegaly and characteristic laboratory findings. HLH may be inherited (primary) or may be secondary to any severe infection, malignancy or rheumatologic condition. The last several years have witnessed an explosion in our understanding of HLH. Of the inherited causes for which the underlying genetic cause is known, most involve abnormalities of proteins important in… CONTINUE READING