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Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran
BACKGROUND: Thrombotic microangiopathy (TMA) is a rare but devastating small vessels disorder that is characterized by intravascular platelet thrombi, thrombocytopenia, and various degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. OBJECTIVES: The Ai...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Kowsar
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4330667/ https://www.ncbi.nlm.nih.gov/pubmed/25738110 http://dx.doi.org/10.5812/numonthly.18900 |
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author | Ardalan, Mohammadreza Rezaeifar, Parisa |
author_facet | Ardalan, Mohammadreza Rezaeifar, Parisa |
author_sort | Ardalan, Mohammadreza |
collection | PubMed |
description | BACKGROUND: Thrombotic microangiopathy (TMA) is a rare but devastating small vessels disorder that is characterized by intravascular platelet thrombi, thrombocytopenia, and various degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. OBJECTIVES: The Aim of this study was to determine the von Willebrand factor-cleaving protease (ADAMTS13) activity during the acute phase of TMA. We also investigated inhibiting antibodies against ADAMTS13 in these patients. PATIENTS AND METHODS: In a collaborative work with Mario-Negro institute of pharmacological research in Bergamo-Italy, we registered the clinical and laboratory data, collected the serum samples, and transferred the samples to the laboratories. Serum samples were taken before the start of plasmapheresis or at least 15 days after the final exchange. RESULTS: We recruited 40 patients (14 males and 26 females) with the mean age of 46.12 ± 17.26 years. The mean activity of ADAMTS13 was 34.58% ± 21.83%. Two patients had inhibitory antibodies against ADAMTS13 with profound deficiency of ADAMTS13 activity (< 6%). Infectious diseases were the most common underlying condition, followed by systemic lupus erythematous. CONCLUSIONS: Majority of patients had an underlying condition and had various ADAMTS13 activity. The presence of inhibiting antibodies and accompanied complete deficiency of ADAMTS13 activity is an indicator of severity. |
format | Online Article Text |
id | pubmed-4330667 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | Kowsar |
record_format | MEDLINE/PubMed |
spelling | pubmed-43306672015-03-03 Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran Ardalan, Mohammadreza Rezaeifar, Parisa Nephrourol Mon Research Article BACKGROUND: Thrombotic microangiopathy (TMA) is a rare but devastating small vessels disorder that is characterized by intravascular platelet thrombi, thrombocytopenia, and various degrees of organ ischemia and anemia, which is due to erythrocyte fragmentation in microcirculation. OBJECTIVES: The Aim of this study was to determine the von Willebrand factor-cleaving protease (ADAMTS13) activity during the acute phase of TMA. We also investigated inhibiting antibodies against ADAMTS13 in these patients. PATIENTS AND METHODS: In a collaborative work with Mario-Negro institute of pharmacological research in Bergamo-Italy, we registered the clinical and laboratory data, collected the serum samples, and transferred the samples to the laboratories. Serum samples were taken before the start of plasmapheresis or at least 15 days after the final exchange. RESULTS: We recruited 40 patients (14 males and 26 females) with the mean age of 46.12 ± 17.26 years. The mean activity of ADAMTS13 was 34.58% ± 21.83%. Two patients had inhibitory antibodies against ADAMTS13 with profound deficiency of ADAMTS13 activity (< 6%). Infectious diseases were the most common underlying condition, followed by systemic lupus erythematous. CONCLUSIONS: Majority of patients had an underlying condition and had various ADAMTS13 activity. The presence of inhibiting antibodies and accompanied complete deficiency of ADAMTS13 activity is an indicator of severity. Kowsar 2014-11-11 /pmc/articles/PMC4330667/ /pubmed/25738110 http://dx.doi.org/10.5812/numonthly.18900 Text en Copyright © 2014, Nephrology and Urology Research Center. http://creativecommons.org/licenses/by-nc/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/) which permits copy and redistribute the material just in noncommercial usages, provided the original work is properly cited. |
spellingShingle | Research Article Ardalan, Mohammadreza Rezaeifar, Parisa Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title | Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title_full | Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title_fullStr | Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title_full_unstemmed | Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title_short | Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Microangiopathy: First Report From Iran |
title_sort | von willebrand factor-cleaving protease activity in thrombotic microangiopathy: first report from iran |
topic | Research Article |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4330667/ https://www.ncbi.nlm.nih.gov/pubmed/25738110 http://dx.doi.org/10.5812/numonthly.18900 |
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