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Lipoprotein Alterations and Plasma Lipoprotein Lipase Reduction in Familial Hemophagocytic Lymphohistiocytosis
Author(s) -
HENTER JANINGE,
CARLSON LARS A.,
SÖDER OLLE,
NILSSONEHLE PETER,
ELINDER GÖRAN
Publication year - 1991
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1991.tb11928.x
Subject(s) - lipoprotein lipase , medicine , hepatosplenomegaly , endocrinology , hepatic lipase , lipoprotein , cholesterol , very low density lipoprotein , adipose tissue , disease
. Serum lipid abnormalities are common in familial hemophagocytic lymphohistocytosis (FHL), a disorder also characterized by fever, hepatosplenomegaly, pancytopenia and a prominent lymphohistiocytic accumulation in the mononuclear phagocyte system. The lipoprotein pattern in nine children with FHL was studied with a quantitative method measuring cholesterol and triglycerides in each major class of lipoproteins. Triglycerides were markedly elevated during active FHL in serum, very low density lipoproteins, and low density lipoproteins. Cholesterol was increased in very low density lipoproteins whereas both triglycerides and cholesterol were extremely low in high density lipoproteins. These lipoprotein abnormalities, reversible on successful therapy, are compatible with a depressed lipolytic activity. Post‐heparin levels of lipoprotein lipase and hepatic lipase in plasma were studied in four children and found to be markedly low during active FHL. We suggest that inflammatory cytokines, which may strongly suppress lipoprotein lipase activity, can be important mediators in the pathophysiology of FHL and that they may participate in the development of the lipid abnormalities.