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Increased fragmentation of von Willebrand factor, due to abnormal cleavage of the subunit, parallels disease activity in recurrent hemolytic uremic syndrome and thrombotic thrombocytopenic purpura and discloses predisposition in families

Academic Article
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Overview

authors

  • Galbusera, M.
  • Noris, M.
  • Rossi, C.
  • Orisio, S.
  • Caprioli, J.
  • Ruggeri, Zaverio
  • Amadei, B.
  • Ruggenenti, P.
  • Vasile, B.
  • Casari, G.
  • Remuzzi, G.
  • Italian Registry of Familial and Recurrent HUS/TTP

publication date

  • July 1999

journal

  • Blood  Journal

abstract

  • We investigated here the changes in von Willebrand factor (vWF) multimers in recurrent, sporadic and familial forms of hemolytic uremic syndrome (HUS)/thrombotic thrombocytopenic purpura (TTP) to see whether they are actually proteolyzed in vivo in these patients. Molecular determinants of fragments in vWF were also characterized to identify possible sites of cleavage of the subunit. Unusually large vWF multimers were found in blood of 8 of 10 patients with recurrent HUS/TTP, both in the acute phase and in remission, but never in familial and sporadic cases. Instead, all of the groups showed evidence of enhanced fragmentation of vWF multimers during the acute phase. Increased fragmentation was also shown by decrease in native 225-kD vWF subunit. In recurrent and sporadic HUS/TTP, enhanced fragmentation normalized at remission, but the abnormality persisted in familial HUS/TTP patients. The latter findings suggest that patients with familial HUS/TTP may have a congenital abnormality in vWF processing. Analysis with specific monoclonal antibodies showed the presence of the normal vWF fragments with apparent molecular mass of 189, 176, and 140 kD in all patients; however, in 6 recurrent and in 5 familial cases, novel fragments that differed in size from normal ones were found. The size of these abnormal fragments differed from one patient to another and none of them was ever found in normal plasma. These results documented, for the first time in HUS/TTP, an abnormal cleavage of the vWF subunit that might account for the increased fragmentation observed in these patients.

subject areas

  • Acute Disease
  • Adolescent
  • Adult
  • Antibodies, Monoclonal
  • Child, Preschool
  • Endopeptidases
  • Female
  • Genetic Predisposition to Disease
  • Hemolytic-Uremic Syndrome
  • Humans
  • Infant
  • Male
  • Middle Aged
  • Peptide Fragments
  • Purpura, Thrombotic Thrombocytopenic
  • Recurrence
  • von Willebrand Factor
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Identity

International Standard Serial Number (ISSN)

  • 0006-4971

PubMed ID

  • 10397728
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Additional Document Info

start page

  • 610

end page

  • 620

volume

  • 94

issue

  • 2

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