Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina

Autores
Dos Santos, Célia; Paiva Palomino, Juvenal Hernán; Romero, María Lucila; Agazzoni, Mara; Kempfer, Ana Catalina; Rotondo, Sabrina; Casinelli, Maria Marta; Alberto, Maria Fabiana; Sánchez Luceros, Analía Gabriela
Año de publicación
2020
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients. Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016. Patients’ subcategory classification relied on clinical symptoms and presence or absence of trigger events associated with TMA. Results: Main suspected disorders were the primary TMAs known as thrombotic thrombocytopenic purpura (TTP) (n = 72/294, 24%) and atypical haemolytic uraemic syndrome (aHUS) (n = 94/294, 32%). In acute phase, demographic parameters for acquired TTP (aTTP) (n = 28) and aHUS (n = 47) showed that both groups were characterised by a young median age (37 and 25 years, respectively) and female predominance (60% and 86%). Median of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 activity was significantly lower in aTTP than in aHUS group (1.4% vs 83%) and was associated with a more severe thrombocytopenia (15 × 109 vs 53 × 109/L). Creatinine (Cr) and urea (Ur) were significantly increased in aHUS compared to aTTP subjects (Cr: 3.7 vs 0.7 mg/dL, Ur: 118 vs 33 mg/dL). Gastrointestinal and neurological symptoms were more frequent in aHUS and aTTP, respectively. Conclusion: The first description of a TMA cohort in Argentina revealed similar clinical presentations to those of other countries.
Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Paiva Palomino, Juvenal Hernán. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Romero, María Lucila. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Agazzoni, Mara. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Kempfer, Ana Catalina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Rotondo, Sabrina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Casinelli, Maria Marta. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Materia
Thrombotic Microangiopathies
thrombotic thrombocytopenic purpura
ADAMTS13
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/146449
Acceder
id CONICETDig_1048dc86a12f1e883225495dc201c3aa
oai_identifier_str oai:ri.conicet.gov.ar:11336/146449
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Thrombotic microangiopathies: First report of 294 cases from a single institution experience in ArgentinaDos Santos, CéliaPaiva Palomino, Juvenal HernánRomero, María LucilaAgazzoni, MaraKempfer, Ana CatalinaRotondo, SabrinaCasinelli, Maria MartaAlberto, Maria FabianaSánchez Luceros, Analía GabrielaThrombotic Microangiopathiesthrombotic thrombocytopenic purpuraADAMTS13https://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients. Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016. Patients’ subcategory classification relied on clinical symptoms and presence or absence of trigger events associated with TMA. Results: Main suspected disorders were the primary TMAs known as thrombotic thrombocytopenic purpura (TTP) (n = 72/294, 24%) and atypical haemolytic uraemic syndrome (aHUS) (n = 94/294, 32%). In acute phase, demographic parameters for acquired TTP (aTTP) (n = 28) and aHUS (n = 47) showed that both groups were characterised by a young median age (37 and 25 years, respectively) and female predominance (60% and 86%). Median of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 activity was significantly lower in aTTP than in aHUS group (1.4% vs 83%) and was associated with a more severe thrombocytopenia (15 × 109 vs 53 × 109/L). Creatinine (Cr) and urea (Ur) were significantly increased in aHUS compared to aTTP subjects (Cr: 3.7 vs 0.7 mg/dL, Ur: 118 vs 33 mg/dL). Gastrointestinal and neurological symptoms were more frequent in aHUS and aTTP, respectively. Conclusion: The first description of a TMA cohort in Argentina revealed similar clinical presentations to those of other countries.Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaFil: Paiva Palomino, Juvenal Hernán. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Romero, María Lucila. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Agazzoni, Mara. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Kempfer, Ana Catalina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaFil: Rotondo, Sabrina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Casinelli, Maria Marta. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; ArgentinaJohn Wiley & Sons Ltd2020-12info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/146449Dos Santos, Célia; Paiva Palomino, Juvenal Hernán; Romero, María Lucila; Agazzoni, Mara; Kempfer, Ana Catalina; et al.; Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina; John Wiley & Sons Ltd; eJHaem; 2; 2; 12-2020; 149-1562688-6146CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/https://onlinelibrary.wiley.com/doi/10.1002/jha2.154info:eu-repo/semantics/altIdentifier/doi/10.1002/jha2.154info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-22T11:24:39Zoai:ri.conicet.gov.ar:11336/146449instacron:CONICETInstitucionalhttp://ri.conicet.gov.ar/Organismo científico-tecnológicoNo correspondehttp://ri.conicet.gov.ar/oai/requestdasensio@conicet.gov.ar; lcarlino@conicet.gov.arArgentinaNo correspondeNo correspondeNo correspondeopendoar:34982025-10-22 11:24:39.947CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
title Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
spellingShingle Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
Dos Santos, Célia
Thrombotic Microangiopathies
thrombotic thrombocytopenic purpura
ADAMTS13
title_short Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
title_full Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
title_fullStr Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
title_full_unstemmed Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
title_sort Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
dc.creator.none.fl_str_mv Dos Santos, Célia
Paiva Palomino, Juvenal Hernán
Romero, María Lucila
Agazzoni, Mara
Kempfer, Ana Catalina
Rotondo, Sabrina
Casinelli, Maria Marta
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author Dos Santos, Célia
author_facet Dos Santos, Célia
Paiva Palomino, Juvenal Hernán
Romero, María Lucila
Agazzoni, Mara
Kempfer, Ana Catalina
Rotondo, Sabrina
Casinelli, Maria Marta
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author_role author
author2 Paiva Palomino, Juvenal Hernán
Romero, María Lucila
Agazzoni, Mara
Kempfer, Ana Catalina
Rotondo, Sabrina
Casinelli, Maria Marta
Alberto, Maria Fabiana
Sánchez Luceros, Analía Gabriela
author2_role author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Thrombotic Microangiopathies
thrombotic thrombocytopenic purpura
ADAMTS13
topic Thrombotic Microangiopathies
thrombotic thrombocytopenic purpura
ADAMTS13
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients. Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016. Patients’ subcategory classification relied on clinical symptoms and presence or absence of trigger events associated with TMA. Results: Main suspected disorders were the primary TMAs known as thrombotic thrombocytopenic purpura (TTP) (n = 72/294, 24%) and atypical haemolytic uraemic syndrome (aHUS) (n = 94/294, 32%). In acute phase, demographic parameters for acquired TTP (aTTP) (n = 28) and aHUS (n = 47) showed that both groups were characterised by a young median age (37 and 25 years, respectively) and female predominance (60% and 86%). Median of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 activity was significantly lower in aTTP than in aHUS group (1.4% vs 83%) and was associated with a more severe thrombocytopenia (15 × 109 vs 53 × 109/L). Creatinine (Cr) and urea (Ur) were significantly increased in aHUS compared to aTTP subjects (Cr: 3.7 vs 0.7 mg/dL, Ur: 118 vs 33 mg/dL). Gastrointestinal and neurological symptoms were more frequent in aHUS and aTTP, respectively. Conclusion: The first description of a TMA cohort in Argentina revealed similar clinical presentations to those of other countries.
Fil: Dos Santos, Célia. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Paiva Palomino, Juvenal Hernán. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Romero, María Lucila. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Agazzoni, Mara. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Kempfer, Ana Catalina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
Fil: Rotondo, Sabrina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Casinelli, Maria Marta. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Alberto, Maria Fabiana. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Sánchez Luceros, Analía Gabriela. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina. Consejo Nacional de Investigaciones Científicas y Técnicas. Instituto de Medicina Experimental. Academia Nacional de Medicina de Buenos Aires. Instituto de Medicina Experimental; Argentina
description Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients. Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016. Patients’ subcategory classification relied on clinical symptoms and presence or absence of trigger events associated with TMA. Results: Main suspected disorders were the primary TMAs known as thrombotic thrombocytopenic purpura (TTP) (n = 72/294, 24%) and atypical haemolytic uraemic syndrome (aHUS) (n = 94/294, 32%). In acute phase, demographic parameters for acquired TTP (aTTP) (n = 28) and aHUS (n = 47) showed that both groups were characterised by a young median age (37 and 25 years, respectively) and female predominance (60% and 86%). Median of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 activity was significantly lower in aTTP than in aHUS group (1.4% vs 83%) and was associated with a more severe thrombocytopenia (15 × 109 vs 53 × 109/L). Creatinine (Cr) and urea (Ur) were significantly increased in aHUS compared to aTTP subjects (Cr: 3.7 vs 0.7 mg/dL, Ur: 118 vs 33 mg/dL). Gastrointestinal and neurological symptoms were more frequent in aHUS and aTTP, respectively. Conclusion: The first description of a TMA cohort in Argentina revealed similar clinical presentations to those of other countries.
publishDate 2020
dc.date.none.fl_str_mv 2020-12
dc.type.none.fl_str_mv info:eu-repo/semantics/article
info:eu-repo/semantics/publishedVersion
http://purl.org/coar/resource_type/c_6501
info:ar-repo/semantics/articulo
format article
status_str publishedVersion
dc.identifier.none.fl_str_mv http://hdl.handle.net/11336/146449
Dos Santos, Célia; Paiva Palomino, Juvenal Hernán; Romero, María Lucila; Agazzoni, Mara; Kempfer, Ana Catalina; et al.; Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina; John Wiley & Sons Ltd; eJHaem; 2; 2; 12-2020; 149-156
2688-6146
CONICET Digital
CONICET
url http://hdl.handle.net/11336/146449
identifier_str_mv Dos Santos, Célia; Paiva Palomino, Juvenal Hernán; Romero, María Lucila; Agazzoni, Mara; Kempfer, Ana Catalina; et al.; Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina; John Wiley & Sons Ltd; eJHaem; 2; 2; 12-2020; 149-156
2688-6146
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/https://onlinelibrary.wiley.com/doi/10.1002/jha2.154
info:eu-repo/semantics/altIdentifier/doi/10.1002/jha2.154
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
dc.publisher.none.fl_str_mv John Wiley & Sons Ltd
publisher.none.fl_str_mv John Wiley & Sons Ltd
dc.source.none.fl_str_mv reponame:CONICET Digital (CONICET)
instname:Consejo Nacional de Investigaciones Científicas y Técnicas
reponame_str CONICET Digital (CONICET)
collection CONICET Digital (CONICET)
instname_str Consejo Nacional de Investigaciones Científicas y Técnicas
repository.name.fl_str_mv CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicas
repository.mail.fl_str_mv dasensio@conicet.gov.ar; lcarlino@conicet.gov.ar
_version_ 1846781787304361984
score 12.982451

Publicaciones similares