Thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome.

Abstract:

:Large and unusually large von Willebrand factor (vWf) multimers may be responsible for systemic platelet aggregation in thrombotic thrombocytopenic purpura (TTP). This possibility is supported by studies that show deficient vWf-cleaving metalloproteinase and increased platelet-vWf binding during TTP episodes. In acute idiopathic TTP, decreased vWf metalloproteinase is the result of autoantibodies against the enzyme. In familial and acquired hemolytic-uremic syndrome, vWf-cleaving metalloproteinase activity is normal. A deficiency or defect in factor H, which normally dampens the activation of C3 via the alternative complement pathway, has been seen in some patients with familial hemolytic-uremic syndrome. Ticlopidine therapy is an important risk factor for TTP.

journal_name

Curr Opin Pediatr

authors

Baker KR,Moake JL

doi

10.1097/00008480-200002000-00005

keywords:

subject

Has Abstract

pub_date

2000-02-01 00:00:00

pages

23-8

issue

1

eissn

1040-8703

issn

1531-698X

journal_volume

12

pub_type

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