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Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient
Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microvascular disorder, which is characterized by pentad of thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction due to occlusive thrombi. The proposed pathophysiology involves an imbalance between unusually large...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Co-Action Publishing
2016
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5161797/ https://www.ncbi.nlm.nih.gov/pubmed/27987277 http://dx.doi.org/10.3402/jchimp.v6.32310 |
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author | Malik, Faizan Ali, Naveed Ahsan, Irfan Ghani, Ali Raza Fidler, Christian |
author_facet | Malik, Faizan Ali, Naveed Ahsan, Irfan Ghani, Ali Raza Fidler, Christian |
author_sort | Malik, Faizan |
collection | PubMed |
description | Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microvascular disorder, which is characterized by pentad of thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction due to occlusive thrombi. The proposed pathophysiology involves an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. Acute pancreatitis is a well-described consequence of TTP, but TTP secondary to acute pancreatitis is a rare phenomenon. We present a patient who developed TTP due to post-ERCP pancreatitis with hematologic, cardiovascular, pulmonary, and renal complications and is the first case of this kind. Despite early initiation of therapy, the patient did not recover making it among the 10% of cases of TTP that prove fatal despite appropriate therapy. |
format | Online Article Text |
id | pubmed-5161797 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Co-Action Publishing |
record_format | MEDLINE/PubMed |
spelling | pubmed-51617972017-01-03 Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient Malik, Faizan Ali, Naveed Ahsan, Irfan Ghani, Ali Raza Fidler, Christian J Community Hosp Intern Med Perspect Case Report Thrombotic thrombocytopenic purpura (TTP) is a rare multisystem microvascular disorder, which is characterized by pentad of thrombocytopenia, microangiopathic hemolytic anemia, and organ dysfunction due to occlusive thrombi. The proposed pathophysiology involves an imbalance between unusually large von Willebrand factor multimers and the cleaving protease ADAMTS13. Acute pancreatitis is a well-described consequence of TTP, but TTP secondary to acute pancreatitis is a rare phenomenon. We present a patient who developed TTP due to post-ERCP pancreatitis with hematologic, cardiovascular, pulmonary, and renal complications and is the first case of this kind. Despite early initiation of therapy, the patient did not recover making it among the 10% of cases of TTP that prove fatal despite appropriate therapy. Co-Action Publishing 2016-12-15 /pmc/articles/PMC5161797/ /pubmed/27987277 http://dx.doi.org/10.3402/jchimp.v6.32310 Text en © 2016 Faizan Malik et al. 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, permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Malik, Faizan Ali, Naveed Ahsan, Irfan Ghani, Ali Raza Fidler, Christian Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title | Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title_full | Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title_fullStr | Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title_full_unstemmed | Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title_short | Eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
title_sort | eculizumab refractory thrombotic thrombocytopenic purpura secondary to post-endoscopic retrograde cholangiopancreatography pancreatitis in a patient |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5161797/ https://www.ncbi.nlm.nih.gov/pubmed/27987277 http://dx.doi.org/10.3402/jchimp.v6.32310 |
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