Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution

Autores
Sánchez Luceros, Analía Gabriela; Meschengieser, Susana S.; Woods, Adriana Inés; Blanco, Alicia Noemi; Kempfer, Ana Catalina; Casais, Patricia; Salviu, Maria J.; Lazzari, María Ángela
Año de publicación
2002
Idioma
inglés
Tipo de recurso
artículo
Estado
versión publicada
Descripción
Background and Objectives. Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. Design and Methods. The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. Results. In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the notMPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. Interpretation and Conclusions. AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests
Fil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Meschengieser, Susana S.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Woods, Adriana Inés. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Blanco, Alicia Noemi. 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; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Casais, Patricia. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Salviu, Maria J.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Lazzari, María Ángela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Materia
Acquired von Willebrand syndrome
myeloproliferative disorders
Nivel de accesibilidad
acceso abierto
Condiciones de uso
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
Repositorio
CONICET Digital (CONICET)
Institución
Consejo Nacional de Investigaciones Científicas y Técnicas
OAI Identificador
oai:ri.conicet.gov.ar:11336/55320
Acceder
id CONICETDig_808121039f6b3160ebb2e4309b857458
oai_identifier_str oai:ri.conicet.gov.ar:11336/55320
network_acronym_str CONICETDig
repository_id_str 3498
network_name_str CONICET Digital (CONICET)
spelling Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single InstitutionSánchez Luceros, Analía GabrielaMeschengieser, Susana S.Woods, Adriana InésBlanco, Alicia NoemiKempfer, Ana CatalinaCasais, PatriciaSalviu, Maria J.Lazzari, María ÁngelaAcquired von Willebrand syndromemyeloproliferative disordershttps://purl.org/becyt/ford/3.2https://purl.org/becyt/ford/3Background and Objectives. Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. Design and Methods. The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. Results. In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the notMPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. Interpretation and Conclusions. AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory testsFil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Meschengieser, Susana S.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Woods, Adriana Inés. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Blanco, Alicia Noemi. 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; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Casais, Patricia. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Salviu, Maria J.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFil: Lazzari, María Ángela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; ArgentinaFerrata Storti Foundation2002-03info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionhttp://purl.org/coar/resource_type/c_6501info:ar-repo/semantics/articuloapplication/pdfapplication/pdfapplication/pdfapplication/pdfapplication/pdfapplication/pdfhttp://hdl.handle.net/11336/55320Sánchez Luceros, Analía Gabriela; Meschengieser, Susana S.; Woods, Adriana Inés; Blanco, Alicia Noemi; Kempfer, Ana Catalina; et al.; Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution; Ferrata Storti Foundation; Haematologica; 87; 3-2002; 264-2700390-60781592-8721CONICET DigitalCONICETenginfo:eu-repo/semantics/altIdentifier/url/http://www.haematologica.org/content/87/3/264info:eu-repo/semantics/openAccesshttps://creativecommons.org/licenses/by-nc-sa/2.5/ar/reponame:CONICET Digital (CONICET)instname:Consejo Nacional de Investigaciones Científicas y Técnicas2025-10-22T11:38:04Zoai:ri.conicet.gov.ar:11336/55320instacron: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 11:38:04.668CONICET Digital (CONICET) - Consejo Nacional de Investigaciones Científicas y Técnicasfalse
dc.title.none.fl_str_mv Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
title Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
spellingShingle Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
Sánchez Luceros, Analía Gabriela
Acquired von Willebrand syndrome
myeloproliferative disorders
title_short Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
title_full Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
title_fullStr Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
title_full_unstemmed Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
title_sort Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution
dc.creator.none.fl_str_mv Sánchez Luceros, Analía Gabriela
Meschengieser, Susana S.
Woods, Adriana Inés
Blanco, Alicia Noemi
Kempfer, Ana Catalina
Casais, Patricia
Salviu, Maria J.
Lazzari, María Ángela
author Sánchez Luceros, Analía Gabriela
author_facet Sánchez Luceros, Analía Gabriela
Meschengieser, Susana S.
Woods, Adriana Inés
Blanco, Alicia Noemi
Kempfer, Ana Catalina
Casais, Patricia
Salviu, Maria J.
Lazzari, María Ángela
author_role author
author2 Meschengieser, Susana S.
Woods, Adriana Inés
Blanco, Alicia Noemi
Kempfer, Ana Catalina
Casais, Patricia
Salviu, Maria J.
Lazzari, María Ángela
author2_role author
author
author
author
author
author
author
dc.subject.none.fl_str_mv Acquired von Willebrand syndrome
myeloproliferative disorders
topic Acquired von Willebrand syndrome
myeloproliferative disorders
purl_subject.fl_str_mv https://purl.org/becyt/ford/3.2
https://purl.org/becyt/ford/3
dc.description.none.fl_txt_mv Background and Objectives. Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. Design and Methods. The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. Results. In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the notMPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. Interpretation and Conclusions. AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests
Fil: Sánchez Luceros, Analía Gabriela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Meschengieser, Susana S.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Woods, Adriana Inés. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Blanco, Alicia Noemi. 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; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Casais, Patricia. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Salviu, Maria J.. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
Fil: Lazzari, María Ángela. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Academia Nacional de Medicina de Buenos Aires. Instituto de Investigaciones Hematológicas "Mariano R. Castex"; Argentina
description Background and Objectives. Acquired von Willebrand syndrome (AVWS) is a rare acquired disorder. In most cases it is associated with lymphoproliferative disorders and monoclonal gammopathies, while less frequently myeloproliferative disorders (MPD) are involved. Although bleeding is the most important symptom, thrombotic complications have also been observed in cases associated with MPD. Our aim was to review the clinical and laboratory findings in AVWS patients from a single institution. Design and Methods. The records of 99 patients with AVWS were reviewed to identify the underlying diseases, the symptoms and the laboratory parameters. Results. In 75% of cases the AVWS was associated with MPD. The most frequent pattern was type 2 (67.7%). Abnormalities of bleeding time, factor VIII levels or platelet retention to glass beads were observed in 83.8% of cases. Bleeding was present in 38.4% of patients, more frequently in the notMPD-associated (58.3%) vs. MPD-associated cases (32%) (p=0.022), with a significant predominance in females, irrespective of the underlying disease (p=0.0007). In 32% of patients with MPD, thrombotic manifestations, mostly microvascular and arterial episodes, were observed. Interpretation and Conclusions. AVWS in MPD seems to be mainly a laboratory diagnosis, without clinical symptoms in most cases, although bleeding as well as ischemic events can be present. In contrast, AVWS in not-MPD-associated cases is most frequently associated with severe bleeding symptoms. Performing appropriate laboratory tests
publishDate 2002
dc.date.none.fl_str_mv 2002-03
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/11336/55320
Sánchez Luceros, Analía Gabriela; Meschengieser, Susana S.; Woods, Adriana Inés; Blanco, Alicia Noemi; Kempfer, Ana Catalina; et al.; Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution; Ferrata Storti Foundation; Haematologica; 87; 3-2002; 264-270
0390-6078
1592-8721
CONICET Digital
CONICET
url http://hdl.handle.net/11336/55320
identifier_str_mv Sánchez Luceros, Analía Gabriela; Meschengieser, Susana S.; Woods, Adriana Inés; Blanco, Alicia Noemi; Kempfer, Ana Catalina; et al.; Acquired Von Willebrand Factor Abnormalities In Myeloproliferative Disorders And Other Hematologic Diseases: A Retrospective Analysis By A Single Institution; Ferrata Storti Foundation; Haematologica; 87; 3-2002; 264-270
0390-6078
1592-8721
CONICET Digital
CONICET
dc.language.none.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv info:eu-repo/semantics/altIdentifier/url/http://www.haematologica.org/content/87/3/264
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
eu_rights_str_mv openAccess
rights_invalid_str_mv https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.format.none.fl_str_mv application/pdf
application/pdf
application/pdf
application/pdf
application/pdf
application/pdf
dc.publisher.none.fl_str_mv Ferrata Storti Foundation
publisher.none.fl_str_mv Ferrata Storti Foundation
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
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score 12.982451

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