ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome

Kód výsledku v 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>

Výsledek na webu

<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>

Jazyk výsledku

angličtina

Název v původním jazyce

ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome

Popis výsledku v původním jazyce

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.

Název v anglickém jazyce

ADAMTS13 kinetics after therapeutic plasma exchange and plasma infusion in patients with Upshaw-Schulman syndrome

Popis výsledku anglicky

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.

Druh

J_imp - Článek v periodiku v databázi Web of Science

CEP obor

—

OECD FORD obor

30205 - Hematology

Projekt

—

Návaznosti

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Rok uplatnění

2019

Kód důvěrnosti údajů

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Název periodika

Journal of clinical apheresis

ISSN

0733-2459

e-ISSN

1098-1101

Svazek periodika

34

Číslo periodika v rámci svazku

1

Stát vydavatele periodika

US - Spojené státy americké

Počet stran výsledku

8

Strana od-do

13-20

Kód UT WoS článku

000455771900002

EID výsledku v databázi Scopus

2-s2.0-85055918422