Anti-Endothelial Cell Antibodies in Thrombotic Thrombocytopenic Purpura and Haemolytic Uraemic Syndrome
Thrombotic thrombocytopenic purpura (TTP) and haemolytic-uraemic syndrome (HUS), are rare and poorly understood disorders involving multiorgan microvascular platelet thrombus formation and microangiopathic haemolytic anaemic (Kaplan et al, 1992; Kaplan 1992; White et al, 1988). Only in HUS has an initiating pathologic stimulus been identified in the form of toxins associated with specific enteric infections (i.e., E Coli, Shigella)(Karmali et al., 1983; Karmali, 1989; Kavi and Wise, 1989). Because of the effectiveness of plasma exchange therapy (Moake, 1991; Rocketal, 1992; Bell et al, 1991) workers have looked for circulating factors that might explain the therapeutic response. Several have been detected, most notably, abnormally large high molecular weight von Willebrand multimers (Rose et al., 1984) a calcium-dependent cysteine protease (Murphy et al, 1987) and a platelet aggregating protein (p37) (Siddiqui et al, 1985). All these factors reflect responses to a damaging stimulus and are implicated in platelet activation, aggregation and deposition (Moake and McPherson, 1989; Moore et al, 1990; Lian et al, 1991).
KeywordsHemolytic Uremic Syndrome Thrombotic Thrombocytopenic Purpura Haemolytic Uraemic Syndrome Haemolytic Uremic Syndrome Plasma Exchange Therapy
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- Editorial., 1991, Antibodies to endothelial cells. Lancet 337 (March 16): 649.Google Scholar
- Foster, P. A. and Anderson, J.C., 1979, Effects of plasma from patients with thrombotic thrombocytopenic purpura on cultured endothelial cells. Blood 54: 240.Google Scholar
- Kaplan, B.S., Trompeter, RS. and Moake, J.L., 1992, Haemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura, 558, New York: Marcel Dekker.Google Scholar
- Kaplan, B.S., 1992, Commentary on the relationship between hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, in Hemolytic Uremic Syndrome and Thrombotic Thrombocytopenic Purpura, Dekker. Kaplan, Trompeter, Moake (eds). 29–39, New York.Google Scholar
- Karmali, M.A., Steele, B.T., Petric, M. and Lim, C.,1983, Sporadic cases of haemolytic-uraemic syndrome associated with faecal cytotoxic and cytotoxin-producing Eschericia Coli in stools: Lancet (i): 619.Google Scholar
- Karmali, M.A., 1989, Infection by Verocytotoxin-producing Eschericia coli. Clin Microbiol Revs 2: 15.Google Scholar
- Kavi, J., Chant, I., Maris, M. and Rose, P., 1987, Cytopathic effect of verotoxin on endothelial cells: Lancet (ii):1035.Google Scholar
- Leung, D.Y.M., Moake, J.L., Havens, P.L., Kim, M. and Pober, J.S., 1988, Lytic anti-endothelial cell antibodies in haemolytic-uraemic syndrome: Lancet (July 23 ): 183.Google Scholar