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A catastrophic seronegative anti-phospholipid syndrome: case and literature review

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Main Author: Pinto, Vanda
Publication Date: 2021
Other Authors: Ministro, Augusto, Carreira, Nuno, Cardoso, Ana, Gonçalves, Catarina Sousa, Henriques, Mickael, Rato, João, Silva, Emanuel, Pedro, Luís M
Format: Article
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10451/50684
Summary: © The Author(s). 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
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spelling A catastrophic seronegative anti-phospholipid syndrome: case and literature reviewAntiphospholipid syndromeAortic thrombosisRenal thrombosis© The Author(s). 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.Background: Antiphospholipid Syndrome (APS) is a multisystemic autoimmune disease characterized by arterial and venous thrombosis and / or obstetric morbidity in the presence of at least one circulating anti-phospholipid antibody. The spectrum of vascular events varies from deep venous thrombosis to catastrophic APS, a rare form characterized by acute multiorgan thrombosis and high mortality. Case report: We present the case of a 32-week pregnant woman arriving in the hospital emergency room with bilateral acute lower limb ischemia. In the obstetric evaluation, fetal death was declared. Computerized Tomography angiography showed pulmonary embolism of both pulmonary arteries, areas of splenic and right renal infarction and multiple arterial and venous thrombosis. The patient underwent urgent caesarean section and axillary-bifemoral bypass. No events registered. In the postoperative period, in an intensive care unit, treatment with rituximab and plasmapheresis were added to anticoagulant therapy. The laboratorial investigation was negative for thrombophilia and autoimmune diseases. Conclusion: Catastrophic APS develops quickly, with multiorgan involvement and high mortality rate. The presented case poses a multidisciplinary challenge, with the surgical approach of extra-anatomical revascularization being less invasive and guaranteeing immediate perfusion of the lower limbs. Although the serological tests were negative for anti-phospholipid antibodies, this case hardly fits into another diagnosis. Therefore, it was treated as a catastrophic APS, having shown a favorable evolution.Springer NatureRepositório da Universidade de LisboaPinto, VandaMinistro, AugustoCarreira, NunoCardoso, AnaGonçalves, Catarina SousaHenriques, MickaelRato, JoãoSilva, EmanuelPedro, Luís M2022-01-04T12:52:09Z20212021-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10451/50684engThromb J. 2021 Dec 20;19(1):10310.1186/s12959-021-00356-w1477-9560info:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2025-03-17T14:39:39Zoai:repositorio.ulisboa.pt:10451/50684Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-29T03:20:12.336837Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv A catastrophic seronegative anti-phospholipid syndrome: case and literature review
title A catastrophic seronegative anti-phospholipid syndrome: case and literature review
spellingShingle A catastrophic seronegative anti-phospholipid syndrome: case and literature review
Pinto, Vanda
Antiphospholipid syndrome
Aortic thrombosis
Renal thrombosis
title_short A catastrophic seronegative anti-phospholipid syndrome: case and literature review
title_full A catastrophic seronegative anti-phospholipid syndrome: case and literature review
title_fullStr A catastrophic seronegative anti-phospholipid syndrome: case and literature review
title_full_unstemmed A catastrophic seronegative anti-phospholipid syndrome: case and literature review
title_sort A catastrophic seronegative anti-phospholipid syndrome: case and literature review
author Pinto, Vanda
author_facet Pinto, Vanda
Ministro, Augusto
Carreira, Nuno
Cardoso, Ana
Gonçalves, Catarina Sousa
Henriques, Mickael
Rato, João
Silva, Emanuel
Pedro, Luís M
author_role author
author2 Ministro, Augusto
Carreira, Nuno
Cardoso, Ana
Gonçalves, Catarina Sousa
Henriques, Mickael
Rato, João
Silva, Emanuel
Pedro, Luís M
author2_role author
author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv Repositório da Universidade de Lisboa
dc.contributor.author.fl_str_mv Pinto, Vanda
Ministro, Augusto
Carreira, Nuno
Cardoso, Ana
Gonçalves, Catarina Sousa
Henriques, Mickael
Rato, João
Silva, Emanuel
Pedro, Luís M
dc.subject.por.fl_str_mv Antiphospholipid syndrome
Aortic thrombosis
Renal thrombosis
topic Antiphospholipid syndrome
Aortic thrombosis
Renal thrombosis
description © The Author(s). 2021. Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
publishDate 2021
dc.date.none.fl_str_mv 2021
2021-01-01T00:00:00Z
2022-01-04T12:52:09Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
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dc.identifier.uri.fl_str_mv http://hdl.handle.net/10451/50684
url http://hdl.handle.net/10451/50684
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv Thromb J. 2021 Dec 20;19(1):103
10.1186/s12959-021-00356-w
1477-9560
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
eu_rights_str_mv openAccess
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dc.publisher.none.fl_str_mv Springer Nature
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instname_str FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia
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reponame_str Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
collection Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
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