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familial hemophagocytic lymphohistiocytosis


Definition: an extremely rare, usually fatal disease of childhood characterized by multiorgan infiltration by activated macrophages and lymphocytes. The disease is often familial and appears to be inherited as an autosomal recessive trait.

Synonym(s): familial erythrophagocytic lymphohistiocytosis

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.