Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos

Background: Thrombotic thrombocytopenic purpura (TTP) is characterized by anemia, thrombocytopenia, neurological and renal involvement of variable severity and it has a dismal prognosis. Platelet-derived von Willebrand Factor-cleaving metalloprotease ADAMTS-13 activity may orient the diagnosis, but...

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Autores principales:	Eymin L,Gonzalo, Andrade A,Maricarmen, Andresen H,Max, Pereira G,Jaime
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2008
Materias:	Plasmapheresis Purpura, thrombotic thrombocytopenic von Willebrand factor
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001200003
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spelling	oai:scielo:S0034-988720080012000032009-03-23Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casosEymin L,GonzaloAndrade A,MaricarmenAndresen H,MaxPereira G,Jaime Plasmapheresis Purpura, thrombotic thrombocytopenic von Willebrand factor Background: Thrombotic thrombocytopenic purpura (TTP) is characterized by anemia, thrombocytopenia, neurological and renal involvement of variable severity and it has a dismal prognosis. Platelet-derived von Willebrand Factor-cleaving metalloprotease ADAMTS-13 activity may orient the diagnosis, but normal levels do not discard it. The most effective therapy thus known is plasmapheresis. Aim: To report the experience in 18 patients with TTP. Material and methods: Retrospective assessment of 11 patients and prospective assessment of seven subjects with TTP, aged 15 to 81 years. Results: All presented with anemia, thrombocytopenia and LDH elevation. Sixteen had neurological symptoms, five had fever, four had macroscopic urinary excretion of pigments, four had petechiae, and two had nosebleeds. Haptoglobin was low in 10 of 11 patients in whom it was measured. ADAMTS-13 had low activity in 15 of 17 patients (in 11, the inhibitor was found). Seventeen patients were treated with plasmapheresis and nine received steroids also. Seven patients died due to shock with respiratory involvement or múltiple organic failure. Conclusions: TTP has heterogeneous modes of presentation. If the diagnosis is strongly suspected, plasmapheresis can be started without laboratory confirmation. An ADAMTS-13 activity below 6% is almost exclusive of TTP .info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.136 n.12 20082008-12-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001200003es10.4067/S0034-98872008001200003
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Plasmapheresis Purpura, thrombotic thrombocytopenic von Willebrand factor
spellingShingle	Plasmapheresis Purpura, thrombotic thrombocytopenic von Willebrand factor Eymin L,Gonzalo Andrade A,Maricarmen Andresen H,Max Pereira G,Jaime Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
description	Background: Thrombotic thrombocytopenic purpura (TTP) is characterized by anemia, thrombocytopenia, neurological and renal involvement of variable severity and it has a dismal prognosis. Platelet-derived von Willebrand Factor-cleaving metalloprotease ADAMTS-13 activity may orient the diagnosis, but normal levels do not discard it. The most effective therapy thus known is plasmapheresis. Aim: To report the experience in 18 patients with TTP. Material and methods: Retrospective assessment of 11 patients and prospective assessment of seven subjects with TTP, aged 15 to 81 years. Results: All presented with anemia, thrombocytopenia and LDH elevation. Sixteen had neurological symptoms, five had fever, four had macroscopic urinary excretion of pigments, four had petechiae, and two had nosebleeds. Haptoglobin was low in 10 of 11 patients in whom it was measured. ADAMTS-13 had low activity in 15 of 17 patients (in 11, the inhibitor was found). Seventeen patients were treated with plasmapheresis and nine received steroids also. Seven patients died due to shock with respiratory involvement or múltiple organic failure. Conclusions: TTP has heterogeneous modes of presentation. If the diagnosis is strongly suspected, plasmapheresis can be started without laboratory confirmation. An ADAMTS-13 activity below 6% is almost exclusive of TTP .
author	Eymin L,Gonzalo Andrade A,Maricarmen Andresen H,Max Pereira G,Jaime
author_facet	Eymin L,Gonzalo Andrade A,Maricarmen Andresen H,Max Pereira G,Jaime
author_sort	Eymin L,Gonzalo
title	Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
title_short	Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
title_full	Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
title_fullStr	Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
title_full_unstemmed	Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos
title_sort	púrpura trombótico trombocitopénico: revisión de la literatura a partir de 18 casos
publisher	Sociedad Médica de Santiago
publishDate	2008
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872008001200003
work_keys_str_mv	AT eyminlgonzalo purpuratromboticotrombocitopenicorevisiondelaliteraturaapartirde18casos AT andradeamaricarmen purpuratromboticotrombocitopenicorevisiondelaliteraturaapartirde18casos AT andresenhmax purpuratromboticotrombocitopenicorevisiondelaliteraturaapartirde18casos AT pereiragjaime purpuratromboticotrombocitopenicorevisiondelaliteraturaapartirde18casos
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Púrpura trombótico trombocitopénico: Revisión de la literatura a partir de 18 casos

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