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Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview

Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune or hereditary thrombotic microangiopathy (TMA) that may be difficult to recognize given the wide spectrum of presenting symptoms. The clinical diagnosis of TTP is based on thrombocytopenia, microangiopathic hemolytic an...

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Autores principales: Pisklakova, Alexandra, Barbir, Joshua, Sambataro, Jan-Paul, Almanzar, Christian, Manji, Faiza
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8027957/
https://www.ncbi.nlm.nih.gov/pubmed/33842174
http://dx.doi.org/10.7759/cureus.13803
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author Pisklakova, Alexandra
Barbir, Joshua
Sambataro, Jan-Paul
Almanzar, Christian
Manji, Faiza
author_facet Pisklakova, Alexandra
Barbir, Joshua
Sambataro, Jan-Paul
Almanzar, Christian
Manji, Faiza
author_sort Pisklakova, Alexandra
collection PubMed
description Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune or hereditary thrombotic microangiopathy (TMA) that may be difficult to recognize given the wide spectrum of presenting symptoms. The clinical diagnosis of TTP is based on thrombocytopenia, microangiopathic hemolytic anemia and is confirmed by a disintegrin-like and metalloproteinase with thrombospondin type one motif, member 13 (ADAMTS13) <10%. However, the latter confirmation is not rapidly available, and treatment is typically initiated based on the degree of clinical suspicion. The PLASMIC score was recently developed to distinguish between TMA patients with and without severe ADAMTS13 deficiency and used as an adjunct in the diagnosis of TTP when the clinical picture is not clear.  Here we present the case of a completely asymptomatic female with no past medical history diagnosed with TTP after evaluation for thrombocytopenia found on a routine wellness visit. A high PLASMIC score was crucial in the decision to initiate treatment given an unusual asymptomatic presentation. 
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spelling pubmed-80279572021-04-09 Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview Pisklakova, Alexandra Barbir, Joshua Sambataro, Jan-Paul Almanzar, Christian Manji, Faiza Cureus Internal Medicine Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening autoimmune or hereditary thrombotic microangiopathy (TMA) that may be difficult to recognize given the wide spectrum of presenting symptoms. The clinical diagnosis of TTP is based on thrombocytopenia, microangiopathic hemolytic anemia and is confirmed by a disintegrin-like and metalloproteinase with thrombospondin type one motif, member 13 (ADAMTS13) <10%. However, the latter confirmation is not rapidly available, and treatment is typically initiated based on the degree of clinical suspicion. The PLASMIC score was recently developed to distinguish between TMA patients with and without severe ADAMTS13 deficiency and used as an adjunct in the diagnosis of TTP when the clinical picture is not clear.  Here we present the case of a completely asymptomatic female with no past medical history diagnosed with TTP after evaluation for thrombocytopenia found on a routine wellness visit. A high PLASMIC score was crucial in the decision to initiate treatment given an unusual asymptomatic presentation.  Cureus 2021-03-10 /pmc/articles/PMC8027957/ /pubmed/33842174 http://dx.doi.org/10.7759/cureus.13803 Text en Copyright © 2021, Pisklakova et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Internal Medicine
Pisklakova, Alexandra
Barbir, Joshua
Sambataro, Jan-Paul
Almanzar, Christian
Manji, Faiza
Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title_full Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title_fullStr Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title_full_unstemmed Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title_short Silent Thrombotic Thrombocytopenic Purpura: PLASMIC, Lessons Learned, and Current Management Overview
title_sort silent thrombotic thrombocytopenic purpura: plasmic, lessons learned, and current management overview
topic Internal Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8027957/
https://www.ncbi.nlm.nih.gov/pubmed/33842174
http://dx.doi.org/10.7759/cureus.13803
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