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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...

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Autores principales: Malik, Faizan, Ali, Naveed, Ahsan, Irfan, Ghani, Ali Raza, Fidler, Christian
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Co-Action Publishing 2016
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.
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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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