Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , |
Tipo de documento: | Relatório |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008 |
Resumo: | Von Willebrand disease (vWD) is the most prevalent hereditary bleeding disorder, affecting 0.6-1.3% of the population. While gastrointestinal bleeding from angiodysplasia is a well-known complication of vWD, the same is not true for Dieulafoys lesions (DLs). We report the case of a 21-year-old black male with type 1 vWD and 2 previous hospital admissions for severe anemia with no visible blood loss. In both episodes, DLs were identified and treated endoscopically, one in the stomach and another in the duodenum. The patient presented to the emergency department in September 2016 with dizziness, fatigue, and again no visible blood loss. He was hemodynamically stable, and laboratory workup showed a hemoglobin level of 3.4 g/dL. After transfusion of packed red blood cells, intravenous iron, and von Willebrand factor/factor VIII concentrate infusions, the patient underwent upper endoscopy and colonoscopy, which were normal. Small-bowel capsule endoscopy showed dark blood and a fresh clot in the proximal jejunum. At this site, push enteroscopy identified a pulsatile vessel with an overlying minimal mucosal defect, consistent with a DL, type 2b of the Yano-Yamamoto classification, which was successfully treated with adrenaline and 2 hemoclips. The patient remains stable after 18 months of follow-up, with a hemoglobin level of 13.2 g/dL. This is a case of recurrent severe occult gastrointestinal bleeding from multiple DL in a young patient with vWD who is otherwise healthy. Three other cases of DL bleeding in the setting of vWD have been reported in the literature, suggesting a possible association between these 2 entities. |
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Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare AssociationVon Willebrand diseaseVon Willebrand factorDieulafoys lesionSmall-bowel capsule endoscopyPush enteroscopyVon Willebrand disease (vWD) is the most prevalent hereditary bleeding disorder, affecting 0.6-1.3% of the population. While gastrointestinal bleeding from angiodysplasia is a well-known complication of vWD, the same is not true for Dieulafoys lesions (DLs). We report the case of a 21-year-old black male with type 1 vWD and 2 previous hospital admissions for severe anemia with no visible blood loss. In both episodes, DLs were identified and treated endoscopically, one in the stomach and another in the duodenum. The patient presented to the emergency department in September 2016 with dizziness, fatigue, and again no visible blood loss. He was hemodynamically stable, and laboratory workup showed a hemoglobin level of 3.4 g/dL. After transfusion of packed red blood cells, intravenous iron, and von Willebrand factor/factor VIII concentrate infusions, the patient underwent upper endoscopy and colonoscopy, which were normal. Small-bowel capsule endoscopy showed dark blood and a fresh clot in the proximal jejunum. At this site, push enteroscopy identified a pulsatile vessel with an overlying minimal mucosal defect, consistent with a DL, type 2b of the Yano-Yamamoto classification, which was successfully treated with adrenaline and 2 hemoclips. The patient remains stable after 18 months of follow-up, with a hemoglobin level of 13.2 g/dL. This is a case of recurrent severe occult gastrointestinal bleeding from multiple DL in a young patient with vWD who is otherwise healthy. Three other cases of DL bleeding in the setting of vWD have been reported in the literature, suggesting a possible association between these 2 entities.Sociedade Portuguesa de Gastrenterologia2019-06-01info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/reporttext/htmlhttp://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008GE-Portuguese Journal of Gastroenterology v.26 n.3 2019reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAPenghttp://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008Cardoso,Mariana FerreiraLourenço,Luís CarvalhoAntunes,MargaridaBranco,Joana Carvalho eSantos,LilianaMartins,AlexandraReis,Jorge A.info:eu-repo/semantics/openAccess2024-02-06T17:33:57Zoai:scielo:S2341-45452019000300008Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T02:36:07.576118Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
title |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
spellingShingle |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association Cardoso,Mariana Ferreira Von Willebrand disease Von Willebrand factor Dieulafoys lesion Small-bowel capsule endoscopy Push enteroscopy |
title_short |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
title_full |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
title_fullStr |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
title_full_unstemmed |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
title_sort |
Recurrent Gastrointestinal Bleeding from Dieulafoys Lesions in a Patient with Type 1 von Willebrand Disease: A Rare Association |
author |
Cardoso,Mariana Ferreira |
author_facet |
Cardoso,Mariana Ferreira Lourenço,Luís Carvalho Antunes,Margarida Branco,Joana Carvalho e Santos,Liliana Martins,Alexandra Reis,Jorge A. |
author_role |
author |
author2 |
Lourenço,Luís Carvalho Antunes,Margarida Branco,Joana Carvalho e Santos,Liliana Martins,Alexandra Reis,Jorge A. |
author2_role |
author author author author author author |
dc.contributor.author.fl_str_mv |
Cardoso,Mariana Ferreira Lourenço,Luís Carvalho Antunes,Margarida Branco,Joana Carvalho e Santos,Liliana Martins,Alexandra Reis,Jorge A. |
dc.subject.por.fl_str_mv |
Von Willebrand disease Von Willebrand factor Dieulafoys lesion Small-bowel capsule endoscopy Push enteroscopy |
topic |
Von Willebrand disease Von Willebrand factor Dieulafoys lesion Small-bowel capsule endoscopy Push enteroscopy |
description |
Von Willebrand disease (vWD) is the most prevalent hereditary bleeding disorder, affecting 0.6-1.3% of the population. While gastrointestinal bleeding from angiodysplasia is a well-known complication of vWD, the same is not true for Dieulafoys lesions (DLs). We report the case of a 21-year-old black male with type 1 vWD and 2 previous hospital admissions for severe anemia with no visible blood loss. In both episodes, DLs were identified and treated endoscopically, one in the stomach and another in the duodenum. The patient presented to the emergency department in September 2016 with dizziness, fatigue, and again no visible blood loss. He was hemodynamically stable, and laboratory workup showed a hemoglobin level of 3.4 g/dL. After transfusion of packed red blood cells, intravenous iron, and von Willebrand factor/factor VIII concentrate infusions, the patient underwent upper endoscopy and colonoscopy, which were normal. Small-bowel capsule endoscopy showed dark blood and a fresh clot in the proximal jejunum. At this site, push enteroscopy identified a pulsatile vessel with an overlying minimal mucosal defect, consistent with a DL, type 2b of the Yano-Yamamoto classification, which was successfully treated with adrenaline and 2 hemoclips. The patient remains stable after 18 months of follow-up, with a hemoglobin level of 13.2 g/dL. This is a case of recurrent severe occult gastrointestinal bleeding from multiple DL in a young patient with vWD who is otherwise healthy. Three other cases of DL bleeding in the setting of vWD have been reported in the literature, suggesting a possible association between these 2 entities. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019-06-01 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/report |
format |
report |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008 |
url |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
http://scielo.pt/scielo.php?script=sci_arttext&pid=S2341-45452019000300008 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
text/html |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Gastrenterologia |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Gastrenterologia |
dc.source.none.fl_str_mv |
GE-Portuguese Journal of Gastroenterology v.26 n.3 2019 reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799137413602213888 |