Hemophagocytic lymphohistiocytosis in newborn and infants

Published: June 9, 2009
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Familial hemophagocytic lymphohistiocytosis (FHL or HLH) is a rare disorder of early infancy with autosomal recessive inheritance and usually fatal outcome. Its frequency has been estimated around 1 in 50.000 children/year in Sweden. The disease was initially reported in 1952 by a pediatrician who already described most of its prominent features, i.e. propensity to fatal outcome following an infectious disease, a lymphoma-like picture, peculiar observation of hypertriglyceridemia and hemophagocytosis. The familial recurrence of this disease clearly pointed to a genetic defect.

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Aricò, M. (2009). Hemophagocytic lymphohistiocytosis in newborn and infants. Hematology Meeting Reports (formerly Haematologica Reports), 2(10). https://doi.org/10.4081/hmr.v2i10.463