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von Willebrand factor mediates increased platelet retention in recurrent thrombotic thrombocytopenic purpura

Karpman, D LU ; Lethagen, S LU ; Kristoffersson, Anne LU ; Isaksson, C. and Holmberg, Lars LU (1997) In Thrombosis and Haemostasis 78(6). p.62-1456
Abstract

The plasma cryoprecipitate of two brothers with recurrent thrombotic thrombocytopenic purpura (TTP) was previously found to mediate increased platelet retention and contain ultra-large von Willebrand factor (vWF) multimers during remissions. We conducted this study to examine if vWF is involved in the increased platelet retention in TTP. Platelet retention decreased when the patients' plasma was incubated with a monoclonal antibody directed to the vWF epitope which interacts with the platelet receptor glycoprotein Ib or when incubated with a Fab-fragment directed to the platelet receptor glycoprotein IIb/IIIa. Replacement of patient vWF with an equivalent concentration of a factor VIII/vWF concentrate containing no ultra-large vWF... (More)

The plasma cryoprecipitate of two brothers with recurrent thrombotic thrombocytopenic purpura (TTP) was previously found to mediate increased platelet retention and contain ultra-large von Willebrand factor (vWF) multimers during remissions. We conducted this study to examine if vWF is involved in the increased platelet retention in TTP. Platelet retention decreased when the patients' plasma was incubated with a monoclonal antibody directed to the vWF epitope which interacts with the platelet receptor glycoprotein Ib or when incubated with a Fab-fragment directed to the platelet receptor glycoprotein IIb/IIIa. Replacement of patient vWF with an equivalent concentration of a factor VIII/vWF concentrate containing no ultra-large vWF multimers was accompanied by a normalization of platelet retention. These results indicate that vWF is involved in the increased platelet retention. Analysis of polymorphic markers in the vWF gene demonstrated that a recessive mutation in this gene is unlikely.

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published
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keywords
Adolescent, Adult, Antibodies, Monoclonal, Blood Platelets, Child, Child, Preschool, Cryoglobulins, Female, Freezing, Genes, Humans, Male, Pedigree, Plasma, Platelet Count, Polymorphism, Genetic, Purpura, Thrombotic Thrombocytopenic, Recurrence, Structure-Activity Relationship, von Willebrand Factor
in
Thrombosis and Haemostasis
volume
78
issue
6
pages
7 pages
publisher
F K Schattauer Verlag Gmbh
external identifiers
  • scopus:0030690339
ISSN
0340-6245
language
English
LU publication?
yes
id
65c9f398-1e7c-4115-a7bd-371ee9aca477
date added to LUP
2017-02-08 16:03:06
date last changed
2017-02-12 04:39:29
@article{65c9f398-1e7c-4115-a7bd-371ee9aca477,
  abstract     = {<p>The plasma cryoprecipitate of two brothers with recurrent thrombotic thrombocytopenic purpura (TTP) was previously found to mediate increased platelet retention and contain ultra-large von Willebrand factor (vWF) multimers during remissions. We conducted this study to examine if vWF is involved in the increased platelet retention in TTP. Platelet retention decreased when the patients' plasma was incubated with a monoclonal antibody directed to the vWF epitope which interacts with the platelet receptor glycoprotein Ib or when incubated with a Fab-fragment directed to the platelet receptor glycoprotein IIb/IIIa. Replacement of patient vWF with an equivalent concentration of a factor VIII/vWF concentrate containing no ultra-large vWF multimers was accompanied by a normalization of platelet retention. These results indicate that vWF is involved in the increased platelet retention. Analysis of polymorphic markers in the vWF gene demonstrated that a recessive mutation in this gene is unlikely.</p>},
  author       = {Karpman, D and Lethagen, S and Kristoffersson, Anne and Isaksson, C. and Holmberg, Lars},
  issn         = {0340-6245},
  keyword      = {Adolescent,Adult,Antibodies, Monoclonal,Blood Platelets,Child,Child, Preschool,Cryoglobulins,Female,Freezing,Genes,Humans,Male,Pedigree,Plasma,Platelet Count,Polymorphism, Genetic,Purpura, Thrombotic Thrombocytopenic,Recurrence,Structure-Activity Relationship,von Willebrand Factor},
  language     = {eng},
  number       = {6},
  pages        = {62--1456},
  publisher    = {F K Schattauer Verlag Gmbh},
  series       = {Thrombosis and Haemostasis},
  title        = {von Willebrand factor mediates increased platelet retention in recurrent thrombotic thrombocytopenic purpura},
  volume       = {78},
  year         = {1997},
}