Endothelial cell activation in thrombotic thrombocytopenic purpura (TTP): a prospective analysis.
It is generally assumed that endothelial cell injury is the primary event in the pathogenesis of thrombotic thrombocytopenic purpura (TTP). In this study, we have determined the extent of vascular perturbation during acute episodes of the disease. We performed a prospective, serial study of nine patients with relapsing TTP during hospitalization and treatment, and assessed the degree of endothelial cell involvement at admission, exacerbation and remission by measurement of von Willebrand factor (VWF) and VWF-propeptide levels. Measurement of both VWF and its propeptide enabled discrimination between acute and chronic perturbation of the endothelium. Elevated levels of both VWF and propeptide were found at admission. These levels decreased immediately upon plasma exchange therapy. However, plasma VWF and propeptide concentrations did not change, even at the time of acute exacerbation. These observations suggest that endothelial cell activation is not the primary event leading to TTP. Vascular perturbation seems to be a consequence, rather than a cause, of the disease.
Romani De Wit
Thalia
T
Department of Plasma Proteins, Sanquin Research at CLB, Amsterdam, The Netherlands.
Fijnheer
Rob
R
Brinkman
Herm-Jan
HJ
Kersting
Sabina
S
Hene
Ronald J
RJ
van Mourik
Jan A
JA
D000208:Acute Disease; D000328:Adult; D015415:Biomarkers / Q000097:blood; D042783:Endothelial Cells / Q000473:pathology*; D004730:Endothelium, Vascular / Q000473:pathology*; D006801:Humans; D008875:Middle Aged; D015999:Multivariate Analysis; D011446:Prospective Studies; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000473:pathology*; D012008:Recurrence; D014841:von Willebrand Factor / Q000032:analysis
D015415:Biomarkers; D014841:von Willebrand Factor