Defective ADAMTS13 synthesis as a possible consequence of NASH in an obese patient with recurrent thrombotic thrombocytopenic purpura
© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd..
OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is a rare and devastating hematologic disorder frequently associated with multiple organ failure and sometimes death. This syndrome is mainly associated with severe deficiency of ADAMTS13, a disintegrin and metalloprotease with thrombospondin (TSP)-1 repeats, cleaving high molecular weight von Willebrand Factor (ULVWF) multimers. Decreased plasma ADAMTS13 activity results in the accumulation of ULVWF multimers with consequent platelet activation. Recently, obesity has been considered as a potential independent risk factor for TTP, but the reason of this association is still unknown.
METHODS AND RESULTS: We describe an unusual case of fatal recurrent TTP in a morbid obese female with non-alcoholic steatohepatitis (NASH) and severe ADAMTS13 activity deficiency due neither to an inhibitory autoantibody nor to a gene mutation.
CONCLUSIONS: Visceral obesity is associated with non-alcoholic fatty liver disease (NAFLD) and NASH: we hypothesized that these conditions can influence ADAMTS13 antigen and activity. In fact, hepatic stellate cells (HSC) are the main producers of ADAMTS13, and a decrease in ADAMTS13 activity has been reported in liver disease.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2014 |
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| Erschienen: |
2014 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:92 |
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| Enthalten in: |
European journal of haematology - 92(2014), 6 vom: 24. Juni, Seite 497-501 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Lombardi, Anna Maria [VerfasserIn] |
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| Links: |
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| Themen: |
ADAM Proteins |
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| Anmerkungen: |
Date Completed 06.01.2015 Date Revised 25.11.2016 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1111/ejh.12273 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM234816988 |
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| 100 | 1 | |a Lombardi, Anna Maria |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Defective ADAMTS13 synthesis as a possible consequence of NASH in an obese patient with recurrent thrombotic thrombocytopenic purpura |
| 264 | 1 | |c 2014 | |
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| 500 | |a Date Revised 25.11.2016 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd. | ||
| 520 | |a OBJECTIVES: Thrombotic thrombocytopenic purpura (TTP) is a rare and devastating hematologic disorder frequently associated with multiple organ failure and sometimes death. This syndrome is mainly associated with severe deficiency of ADAMTS13, a disintegrin and metalloprotease with thrombospondin (TSP)-1 repeats, cleaving high molecular weight von Willebrand Factor (ULVWF) multimers. Decreased plasma ADAMTS13 activity results in the accumulation of ULVWF multimers with consequent platelet activation. Recently, obesity has been considered as a potential independent risk factor for TTP, but the reason of this association is still unknown | ||
| 520 | |a METHODS AND RESULTS: We describe an unusual case of fatal recurrent TTP in a morbid obese female with non-alcoholic steatohepatitis (NASH) and severe ADAMTS13 activity deficiency due neither to an inhibitory autoantibody nor to a gene mutation | ||
| 520 | |a CONCLUSIONS: Visceral obesity is associated with non-alcoholic fatty liver disease (NAFLD) and NASH: we hypothesized that these conditions can influence ADAMTS13 antigen and activity. In fact, hepatic stellate cells (HSC) are the main producers of ADAMTS13, and a decrease in ADAMTS13 activity has been reported in liver disease | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a ADAMTS13 | |
| 650 | 4 | |a non-alcoholic steatohepatitis | |
| 650 | 4 | |a obesity | |
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| 700 | 1 | |a Fabris, Roberto |e verfasserin |4 aut | |
| 700 | 1 | |a Berti de Marinis, Giulia |e verfasserin |4 aut | |
| 700 | 1 | |a Marson, Piero |e verfasserin |4 aut | |
| 700 | 1 | |a Navaglia, Filippo |e verfasserin |4 aut | |
| 700 | 1 | |a Plebani, Mario |e verfasserin |4 aut | |
| 700 | 1 | |a Vettor, Roberto |e verfasserin |4 aut | |
| 700 | 1 | |a Fabris, Fabrizio |e verfasserin |4 aut | |
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