ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00843989%3A_____%2F19%3AE0107729" target="_blank" >RIV/00843989:_____/19:E0107729 - isvavai.cz</a>
Result on the web
<a href="https://onlinelibrary.wiley.com/doi/pdf/10.1002/jca.21664" target="_blank" >https://onlinelibrary.wiley.com/doi/pdf/10.1002/jca.21664</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1002/jca.21664" target="_blank" >10.1002/jca.21664</a>
Alternative languages
Result language
angličtina
Original language name
ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome
Original language description
Background: Hereditary thrombotic thrombocytopenic purpura, also called Upshaw-Schulman syndrome (USS), is a rare disease caused by genetic mutations in the ADAMTS13 gene, which severely decrease the activity of ADAMTS13, a metalloprotease that cleaves von Willebrand factor multimers (VWF). Genotypically identical patients can show great phenotypic diversity. Objectives: Comparison of selected laboratory parameters and ADAMTS13 pharmacokinetics among patients with USS was performed. Patients/methods Six patients with USS on prophylactic plasma therapy have been reviewed, retrospectively. Blood counts, lactate dehydrogenase (LDH), and ADAMTS13 activity at various time-points before and after different treatment cycles were evaluated. Results: ADAMTS13 recovery and pharmacokinetics were affected by treatment modality, and also reflected the patients' comorbidities and their current physiological and clinical condition. Conclusions: Our present findings support a multifactorial contribution to treatment efficacy, and confirm the importance of adaptability and individualization of USS therapy. Therapeutic plasma exchange even in hereditary TTP is an option that can in some patients prolong intervals between plasma administration.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30205 - Hematology
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2019
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of clinical apheresis
ISSN
0733-2459
e-ISSN
1098-1101
Volume of the periodical
34
Issue of the periodical within the volume
1
Country of publishing house
US - UNITED STATES
Number of pages
8
Pages from-to
13-20
UT code for WoS article
000455771900002
EID of the result in the Scopus database
2-s2.0-85055918422