Thrombotic thrombocytopenic purpura: a case report

ABSTRACT Thrombocytopenic thrombotic purpura (TTP) is a severe hemorrhagic syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia and microvascular occlusion, besides the associated symptoms that may or may not be present: fever, neurological and renal impairment. The pathophysiology involves the autoimmune or genetic deficiency of a metalloproteinases activity (ADAMTS-13), responsible for the von Willebrand Factor cleavage. The treatment is based on plasmapheresis; and in acute or recurrent cases, corticosteroids and immunosuppressants are associated. In this article, we will discuss a case report about this disease, initially treated in the Emergency Room and followed in the Intensive Care Unit of a public reference hospital in São Paulo city, Brazil. All clinical diagnostic criteria were completely filled, facilitating the therapeutic approach of the patient. The report evidences that rapid intervention when made early diagnosis evolves with a good prognosis, and this pathology must be present as a differential diagnosis in the medical routine.

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Main Authors: Drumond,João Paulo N., Abou-Arabi,Rima M., Figueiredo,Raissa P. C., Mourão,Vanessa N., Aiziro,Juliana B., Caly,Décio N., Cortez,Afonso José P.
Format: Digital revista
Language:English
Published: Sociedade Brasileira de Patologia Clínica 2018
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442018000400255
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spelling oai:scielo:S1676-244420180004002552018-09-20Thrombotic thrombocytopenic purpura: a case reportDrumond,João Paulo N.Abou-Arabi,Rima M.Figueiredo,Raissa P. C.Mourão,Vanessa N.Aiziro,Juliana B.Caly,Décio N.Cortez,Afonso José P. purpura thrombotic thrombocytopenic anemia hemolytic plasmapheresis ABSTRACT Thrombocytopenic thrombotic purpura (TTP) is a severe hemorrhagic syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia and microvascular occlusion, besides the associated symptoms that may or may not be present: fever, neurological and renal impairment. The pathophysiology involves the autoimmune or genetic deficiency of a metalloproteinases activity (ADAMTS-13), responsible for the von Willebrand Factor cleavage. The treatment is based on plasmapheresis; and in acute or recurrent cases, corticosteroids and immunosuppressants are associated. In this article, we will discuss a case report about this disease, initially treated in the Emergency Room and followed in the Intensive Care Unit of a public reference hospital in São Paulo city, Brazil. All clinical diagnostic criteria were completely filled, facilitating the therapeutic approach of the patient. The report evidences that rapid intervention when made early diagnosis evolves with a good prognosis, and this pathology must be present as a differential diagnosis in the medical routine.info:eu-repo/semantics/openAccessSociedade Brasileira de Patologia Clínica Jornal Brasileiro de Patologia e Medicina Laboratorial v.54 n.4 20182018-07-01info:eu-repo/semantics/reporttext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442018000400255en10.5935/1676-2444.20180045
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author Drumond,João Paulo N.
Abou-Arabi,Rima M.
Figueiredo,Raissa P. C.
Mourão,Vanessa N.
Aiziro,Juliana B.
Caly,Décio N.
Cortez,Afonso José P.
spellingShingle Drumond,João Paulo N.
Abou-Arabi,Rima M.
Figueiredo,Raissa P. C.
Mourão,Vanessa N.
Aiziro,Juliana B.
Caly,Décio N.
Cortez,Afonso José P.
Thrombotic thrombocytopenic purpura: a case report
author_facet Drumond,João Paulo N.
Abou-Arabi,Rima M.
Figueiredo,Raissa P. C.
Mourão,Vanessa N.
Aiziro,Juliana B.
Caly,Décio N.
Cortez,Afonso José P.
author_sort Drumond,João Paulo N.
title Thrombotic thrombocytopenic purpura: a case report
title_short Thrombotic thrombocytopenic purpura: a case report
title_full Thrombotic thrombocytopenic purpura: a case report
title_fullStr Thrombotic thrombocytopenic purpura: a case report
title_full_unstemmed Thrombotic thrombocytopenic purpura: a case report
title_sort thrombotic thrombocytopenic purpura: a case report
description ABSTRACT Thrombocytopenic thrombotic purpura (TTP) is a severe hemorrhagic syndrome characterized by thrombocytopenia, microangiopathic hemolytic anemia and microvascular occlusion, besides the associated symptoms that may or may not be present: fever, neurological and renal impairment. The pathophysiology involves the autoimmune or genetic deficiency of a metalloproteinases activity (ADAMTS-13), responsible for the von Willebrand Factor cleavage. The treatment is based on plasmapheresis; and in acute or recurrent cases, corticosteroids and immunosuppressants are associated. In this article, we will discuss a case report about this disease, initially treated in the Emergency Room and followed in the Intensive Care Unit of a public reference hospital in São Paulo city, Brazil. All clinical diagnostic criteria were completely filled, facilitating the therapeutic approach of the patient. The report evidences that rapid intervention when made early diagnosis evolves with a good prognosis, and this pathology must be present as a differential diagnosis in the medical routine.
publisher Sociedade Brasileira de Patologia Clínica
publishDate 2018
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1676-24442018000400255
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