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J I Henter

Publications and source records attributed to J I Henter.

59 records · Page 4Linked to original sources
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Lipoprotein alterations and plasma lipoprotein lipase reduction in familial hemophagocytic lymphohistiocytosis.

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.

Child, Preschool↗
PubMedDate not supplied

Myelodysplastic syndrome following epipodophyllotoxin therapy in familial hemophagocytic lymphohistiocytosis.

The prognosis for patients with familial hemophagocytic lymphohistiocytosis (FHL) is poor, but the survival of affected children has been markedly prolonged by treatment with the epipodophyllotoxin derivatives etoposide and teniposide and by bone marrow transplantation. Secondary malignancies following epipodophyllotoxin therapy, including myelodysplastic syndrome (MDS) and acute myelocytic leukemia (AML), have recently been reported. We describe a 9-year-old boy, treated with epipodophyllotoxins for FHL since he was 3 years old, who developed MDS. He was administered etoposide (cumulative doses of 6.9 g/m2 intravenously and 13.6 g/m2 orally) and teniposide (3.4 g/m2 intravenously), but no other systemic antineoplastic drugs. This is, to our knowledge, the first report of a child with FHL developing MDS or AML. Moreover, MDS or AML following administration of epipodophyllotoxins as the sole systemic chemotherapeutic drug has not been reported previously. Supportive treatments, including the use of immunomodulating drugs, may reduce the risk for secondary leukemia in patients with FHL.

Child↗