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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...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2021
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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. |
format | Online Article Text |
id | pubmed-8027957 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | Cureus |
record_format | MEDLINE/PubMed |
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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