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Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise

Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications...

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Autores principales: Skeith, Leslie, Hurd, Kelle, Chaturvedi, Shruti, Chow, Lorraine, Nicholas, Joshua, Lee, Adrienne, Young, Daniel, Goodyear, Dawn, Soucie, Jennifer, Girard, Louis, Dufour, Antoine, Agbani, Ejaife O.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
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Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9737388/
https://www.ncbi.nlm.nih.gov/pubmed/36498688
http://dx.doi.org/10.3390/jcm11237115
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author Skeith, Leslie
Hurd, Kelle
Chaturvedi, Shruti
Chow, Lorraine
Nicholas, Joshua
Lee, Adrienne
Young, Daniel
Goodyear, Dawn
Soucie, Jennifer
Girard, Louis
Dufour, Antoine
Agbani, Ejaife O.
author_facet Skeith, Leslie
Hurd, Kelle
Chaturvedi, Shruti
Chow, Lorraine
Nicholas, Joshua
Lee, Adrienne
Young, Daniel
Goodyear, Dawn
Soucie, Jennifer
Girard, Louis
Dufour, Antoine
Agbani, Ejaife O.
author_sort Skeith, Leslie
collection PubMed
description Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. Methods: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. Results: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. Conclusions: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise.
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spelling pubmed-97373882022-12-11 Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise Skeith, Leslie Hurd, Kelle Chaturvedi, Shruti Chow, Lorraine Nicholas, Joshua Lee, Adrienne Young, Daniel Goodyear, Dawn Soucie, Jennifer Girard, Louis Dufour, Antoine Agbani, Ejaife O. J Clin Med Case Report Background: Congenital thrombotic thrombocytopenic purpura (cTTP) is a rare disorder caused by an inherited genetic deficiency of ADAMTS13 and affects less than one per million individuals. Patients who are diagnosed with TTP during pregnancy are at increased risk of maternal and fetal complications including fetal demise. We present a case of a 32-year-old G3P0 (gravida 3, para 0) who presented at 20 weeks gestation with a new diagnosis of congenital TTP (cTTP) and fetal demise. Methods: We describe the pathophysiology of pregnancy complications in a patient with cTTP using platelet procoagulant membrane dynamics analysis and quantitative proteomic studies, compared to four pregnant patients with gestational hypertension, four pregnant patients with preeclampsia, and four healthy pregnant controls. Results: The cTTP patient had increased P-selectin, tissue factor expression, annexin-V binding on platelets and neutrophils, and localized thrombin generation, suggestive of hypercoagulability. Among 15 proteins that were upregulated, S100A8 and S100A9 were distinctly overexpressed. Conclusions: There is platelet-neutrophil activation and interaction, platelet hypercoagulability, and proinflammation in our case of cTTP with fetal demise. MDPI 2022-11-30 /pmc/articles/PMC9737388/ /pubmed/36498688 http://dx.doi.org/10.3390/jcm11237115 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Case Report
Skeith, Leslie
Hurd, Kelle
Chaturvedi, Shruti
Chow, Lorraine
Nicholas, Joshua
Lee, Adrienne
Young, Daniel
Goodyear, Dawn
Soucie, Jennifer
Girard, Louis
Dufour, Antoine
Agbani, Ejaife O.
Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title_full Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title_fullStr Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title_full_unstemmed Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title_short Hypercoagulability and Inflammatory Markers in a Case of Congenital Thrombotic Thrombocytopenic Purpura Complicated by Fetal Demise
title_sort hypercoagulability and inflammatory markers in a case of congenital thrombotic thrombocytopenic purpura complicated by fetal demise
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9737388/
https://www.ncbi.nlm.nih.gov/pubmed/36498688
http://dx.doi.org/10.3390/jcm11237115
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