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Hypercytokinemia in familial hemophagocytic lymphohistiocytosis
JI Henter, G Elinder, O Soder, M Hansson, B Andersson and U Andersson
Department of Pediatrics, Karolinska Institute, St Goran's Children's
Hospital, Stockholm, Sweden.
Familial hemophagocytic lymphohistiocytosis (FHL) is a frequently missed
and almost uniformly fatal childhood disorder. It is characterized by
fever, hepatosplenomegaly, cytopenia, coagulopathy, and
hypertriglyceridemia. The pathogenesis of FHL is not known but the above
clinical and laboratory findings are compatible with reported in vitro and
in vivo effects of several inflammatory cytokines. We measured circulating
interferon-gamma (IFN-gamma), tumor necrosis factor/cachectin (TNF), and
interleukin-6 (IL-6) in nine children with FHL. During active disease,
elevated IFN-gamma was detected in seven of seven children, TNF in six of
six, and IL-6 in two of six children studied. Thus, important inflammatory
cytokines are augmented in active FHL and may contribute to the
pathogenesis of the disease. Soluble CD8 was also increased in seven of
seven children, which suggests a pathophysiologic importance of cytotoxic T
lymphocytes. Because FHL appears to be associated with a systemic
hypercytokinemia, our results also indicate that studies of FHL may
contribute to the understanding of cytokine effects in vivo. Moreover, FHL
is a hereditary disorder, suggesting that the hypercytokinemia is caused by
a genetic defect in cytokine regulation.
Volume 78,
Issue 11,
pp. 2918-2922,
12/01/1991
Copyright © 1991 by The American Society of Hematology
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