Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case

Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case

We report a 23 years old female who presented a second episode of thrombotic thrombocytopenic purpura (TTP). She was treated with fresh frozen plasma infusions and 14 plasma exchange (PE) sessions without response. Therefore a second-line therapy was started, associating a weekly cycle administratio...

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Autores principales: Ojeda-Uribe,Mario, Brunot,Annick, Issler,Monique
Lenguaje:Spanish / Castilian
Publicado: Sociedad Médica de Santiago 2005
Materias:
ADAMTS13 protein
human
Metalloendopeptidases
Purpura
thrombotic thrombocytopenic
Rituximab
Vindesine
Acceso en línea:http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005001100011
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spelling oai:scielo:S0034-988720050011000112006-01-24Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one caseOjeda-Uribe,MarioBrunot,AnnickIssler,Monique ADAMTS13 protein human Metalloendopeptidases Purpura thrombotic thrombocytopenic Rituximab Vindesine We report a 23 years old female who presented a second episode of thrombotic thrombocytopenic purpura (TTP). She was treated with fresh frozen plasma infusions and 14 plasma exchange (PE) sessions without response. Therefore a second-line therapy was started, associating a weekly cycle administration of vindesine (Vds) 2 mg/m² and rituximab (R) 375 mg/m². Five cycles of this association plus one cycle of R exclusively, were administered. After the third course, biological signs of improvement were observed and complete normalization of blood cell counts and other specific parameters was seen after 8 weeks. From the beginning of her second relapse we detected a severe deficit (<5%) in von Willebrand-cleaving factor (ADAMTS13) associated to the presence of ADAMTS13 inhibitors. The combined treatment induced an improvement in ADAMTS13 values without detectable inhibitors. After 21 months of follow-up the patient was well, without signs of relapse but ADAMTS13 values were still under normal, which may be an unfavorable prognostic factor. PE is the treatment of choice for acquired idiopathic TTP, but for refractory cases or TTP cases with severe ADAMTS13 values/high inhibitor titers, PE associated to an immunosuppressive treatment should be consideredinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.133 n.11 20052005-11-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005001100011es10.4067/S0034-98872005001100011
institution Scielo Chile
collection Scielo Chile
language Spanish / Castilian
topic ADAMTS13 protein
human
Metalloendopeptidases
Purpura
thrombotic thrombocytopenic
Rituximab
Vindesine
spellingShingle ADAMTS13 protein
human
Metalloendopeptidases
Purpura
thrombotic thrombocytopenic
Rituximab
Vindesine
Ojeda-Uribe,Mario
Brunot,Annick
Issler,Monique
Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
description We report a 23 years old female who presented a second episode of thrombotic thrombocytopenic purpura (TTP). She was treated with fresh frozen plasma infusions and 14 plasma exchange (PE) sessions without response. Therefore a second-line therapy was started, associating a weekly cycle administration of vindesine (Vds) 2 mg/m² and rituximab (R) 375 mg/m². Five cycles of this association plus one cycle of R exclusively, were administered. After the third course, biological signs of improvement were observed and complete normalization of blood cell counts and other specific parameters was seen after 8 weeks. From the beginning of her second relapse we detected a severe deficit (<5%) in von Willebrand-cleaving factor (ADAMTS13) associated to the presence of ADAMTS13 inhibitors. The combined treatment induced an improvement in ADAMTS13 values without detectable inhibitors. After 21 months of follow-up the patient was well, without signs of relapse but ADAMTS13 values were still under normal, which may be an unfavorable prognostic factor. PE is the treatment of choice for acquired idiopathic TTP, but for refractory cases or TTP cases with severe ADAMTS13 values/high inhibitor titers, PE associated to an immunosuppressive treatment should be considered
author Ojeda-Uribe,Mario
Brunot,Annick
Issler,Monique
author_facet Ojeda-Uribe,Mario
Brunot,Annick
Issler,Monique
author_sort Ojeda-Uribe,Mario
title Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
title_short Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
title_full Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
title_fullStr Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
title_full_unstemmed Eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (PTT) adquirido idiopático refractario: Report of one case
title_sort eficacia de la asociación rituximab-vindesina en el tratamiento de un caso de púrpura trombótico-trombopénico (ptt) adquirido idiopático refractario: report of one case
publisher Sociedad Médica de Santiago
publishDate 2005
url http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872005001100011
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AT brunotannick eficaciadelaasociacionrituximabvindesinaeneltratamientodeuncasodepurpuratromboticotrombopenicopttadquiridoidiopaticorefractarioreportofonecase
AT isslermonique eficaciadelaasociacionrituximabvindesinaeneltratamientodeuncasodepurpuratromboticotrombopenicopttadquiridoidiopaticorefractarioreportofonecase
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