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Increased platelet retention in familial recurrent thrombotic thrombocytopenic purpura

Karpman, D LU orcid ; Holmberg, Lars LU ; Jirgård, Lena and Lethagen, S LU (1996) In Kidney International 49(1). p.9-190
Abstract

We studied two brothers with recurrent thrombotic thrombocytopenic purpura (TTP). Platelet retention, measured with a modified Adeplat S glass-bead test, was found to be increased during acute episodes of TTP and during remissions. Values of platelet retention ranged between 57 to 95% (normal range 16 to 34%). The continually elevated values enabled us to investigate which fraction of the patients' blood was responsible for the increased platelet retention and to evaluate the effect of different treatments on this parameter. We found that the patients' plasma increased the retention of normal platelets and of platelets taken from a patient with von Willebrand's disease type III. This activity was located in the cryoprecipitate fraction... (More)

We studied two brothers with recurrent thrombotic thrombocytopenic purpura (TTP). Platelet retention, measured with a modified Adeplat S glass-bead test, was found to be increased during acute episodes of TTP and during remissions. Values of platelet retention ranged between 57 to 95% (normal range 16 to 34%). The continually elevated values enabled us to investigate which fraction of the patients' blood was responsible for the increased platelet retention and to evaluate the effect of different treatments on this parameter. We found that the patients' plasma increased the retention of normal platelets and of platelets taken from a patient with von Willebrand's disease type III. This activity was located in the cryoprecipitate fraction of plasma. Unusually large von Willebrand factor (vWF) multimers were demonstrated in both children during remission and decreased during relapse. Both fresh frozen plasma (FFP) and a commercial factor VIII/vWF concentrate reduced platelet retention when tested during remission. Treatment of both siblings with FFP or factor VIII/vWF concentrate was beneficial during recurrences. We conclude that the elevated platelet retention is due to a factor in the cryoprecipitate of the childrens' plasma, and that both FFP and factor VIII/vWF concentrate are effective in decreasing platelet retention.

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author
; ; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Adult, Biomarkers, Blood Platelets, Child, Child, Preschool, Factor VIII, Female, Humans, Male, Nuclear Family, Plasma Exchange, Purpura, Thrombotic Thrombocytopenic, Recurrence, Remission Induction, von Willebrand Diseases, von Willebrand Factor, Journal Article, Research Support, Non-U.S. Gov't
in
Kidney International
volume
49
issue
1
pages
10 pages
publisher
Nature Publishing Group
external identifiers
  • scopus:0030042628
  • pmid:8770967
ISSN
0085-2538
DOI
10.1038/ki.1996.26
language
English
LU publication?
yes
id
b2314748-10e9-4062-bc11-9cd11ed3af54
date added to LUP
2017-02-08 16:05:56
date last changed
2025-01-07 06:32:27
@article{b2314748-10e9-4062-bc11-9cd11ed3af54,
  abstract     = {{<p>We studied two brothers with recurrent thrombotic thrombocytopenic purpura (TTP). Platelet retention, measured with a modified Adeplat S glass-bead test, was found to be increased during acute episodes of TTP and during remissions. Values of platelet retention ranged between 57 to 95% (normal range 16 to 34%). The continually elevated values enabled us to investigate which fraction of the patients' blood was responsible for the increased platelet retention and to evaluate the effect of different treatments on this parameter. We found that the patients' plasma increased the retention of normal platelets and of platelets taken from a patient with von Willebrand's disease type III. This activity was located in the cryoprecipitate fraction of plasma. Unusually large von Willebrand factor (vWF) multimers were demonstrated in both children during remission and decreased during relapse. Both fresh frozen plasma (FFP) and a commercial factor VIII/vWF concentrate reduced platelet retention when tested during remission. Treatment of both siblings with FFP or factor VIII/vWF concentrate was beneficial during recurrences. We conclude that the elevated platelet retention is due to a factor in the cryoprecipitate of the childrens' plasma, and that both FFP and factor VIII/vWF concentrate are effective in decreasing platelet retention.</p>}},
  author       = {{Karpman, D and Holmberg, Lars and Jirgård, Lena and Lethagen, S}},
  issn         = {{0085-2538}},
  keywords     = {{Adult; Biomarkers; Blood Platelets; Child; Child, Preschool; Factor VIII; Female; Humans; Male; Nuclear Family; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Recurrence; Remission Induction; von Willebrand Diseases; von Willebrand Factor; Journal Article; Research Support, Non-U.S. Gov't}},
  language     = {{eng}},
  number       = {{1}},
  pages        = {{9--190}},
  publisher    = {{Nature Publishing Group}},
  series       = {{Kidney International}},
  title        = {{Increased platelet retention in familial recurrent thrombotic thrombocytopenic purpura}},
  url          = {{http://dx.doi.org/10.1038/ki.1996.26}},
  doi          = {{10.1038/ki.1996.26}},
  volume       = {{49}},
  year         = {{1996}},
}