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Lytic anti-endothelial cell antibodies in haemolytic-uraemic syndrome.
Lancet.Jul 23, 1988;2(8604):183-6.
Leung DY, Moake JL, Havens PL, Kim M, Pober JS.
Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Sera from 13 of 14 children with acute haemolytic uraemic syndrome (HUS) contained complement-fixing IgG and IgM antibodies that lysed cultured human umbilical vein endothelial cells. In 3 of 3 sera tested, no lysis of dermal fibroblasts was observed. The endothelial cell antigen was lost after treatment of the cells with gamma interferon. In contrast, only 3 of 5 adult patients with acute, non-relapsing, thrombotic thrombocytopenic purpura (TTP) had lytic anti-endothelial antibodies and only 1 of these recognised an antigen lost upon gamma interferon treatment. None of 32 control sera contained lytic anti-endothelial cell antibodies. These data suggest that HUS involves a disorder of immunoregulation and that a unique class of anti-endothelial cell antibodies is produced that may take part in the pathogenesis of vascular injury in HUS.