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Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome

PURPOSE OF REVIEW: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlig...

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Autores principales: Vreede, Andrew P., Bockenstedt, Paula L., McCune, W. Joseph, Knight, Jason S.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams And Wilkins 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455093/
https://www.ncbi.nlm.nih.gov/pubmed/30747734
http://dx.doi.org/10.1097/BOR.0000000000000595
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author Vreede, Andrew P.
Bockenstedt, Paula L.
McCune, W. Joseph
Knight, Jason S.
author_facet Vreede, Andrew P.
Bockenstedt, Paula L.
McCune, W. Joseph
Knight, Jason S.
author_sort Vreede, Andrew P.
collection PubMed
description PURPOSE OF REVIEW: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding – and persistent knowledge gaps – through clinically oriented questions and answers. RECENT FINDINGS: A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Although the pathophysiology underlying thrombocytopenia in APS has yet to be definitively revealed, mechanisms that play a role (at least in subsets of patients) include: immune thrombocytopenic purpura/ITP-like autoantibodies against platelet glycoproteins; antiphospholipid antibody (aPL)-mediated platelet activation and consumption; and potentially life threatening thrombotic microangiopathy. Although thrombocytopenia is often ‘mild’ in APS (and therefore, may not require specific therapy), there are causes of acute-onset thrombocytopenia that mandate emergent work-up and treatment. When APS-related thrombocytopenia does require therapy, the approach must be individualized (requiring an understanding of pathophysiology in the particular APS patient). For patients with ITP-like disease, rituximab is emerging as a popular approach to treatment; in contrast, there are hints that thrombopoietin mimetics may be associated with elevated thrombotic risk. SUMMARY: Thrombocytopenia is common in APS, and is likely associated with more severe disease. Improved understanding of thrombocytopenia in APS has the potential to improve risk stratification, reveal novel aspects of APS pathophysiology, and lead to treatments that are more individualized and holistic.
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spelling pubmed-64550932019-05-01 Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome Vreede, Andrew P. Bockenstedt, Paula L. McCune, W. Joseph Knight, Jason S. Curr Opin Rheumatol CLINICAL THERAPEUTICS & HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune PURPOSE OF REVIEW: Although antiphospholipid syndrome (APS) is best known for conveying increased risk of thrombotic events and pregnancy morbidity, thrombocytopenia is also recognized as a common association. In this review, we will explore the relationship between thrombocytopenia and APS, highlighting our evolving understanding – and persistent knowledge gaps – through clinically oriented questions and answers. RECENT FINDINGS: A history of thrombocytopenia likely portends a more severe APS phenotype (including increased risk of thrombosis). Although the pathophysiology underlying thrombocytopenia in APS has yet to be definitively revealed, mechanisms that play a role (at least in subsets of patients) include: immune thrombocytopenic purpura/ITP-like autoantibodies against platelet glycoproteins; antiphospholipid antibody (aPL)-mediated platelet activation and consumption; and potentially life threatening thrombotic microangiopathy. Although thrombocytopenia is often ‘mild’ in APS (and therefore, may not require specific therapy), there are causes of acute-onset thrombocytopenia that mandate emergent work-up and treatment. When APS-related thrombocytopenia does require therapy, the approach must be individualized (requiring an understanding of pathophysiology in the particular APS patient). For patients with ITP-like disease, rituximab is emerging as a popular approach to treatment; in contrast, there are hints that thrombopoietin mimetics may be associated with elevated thrombotic risk. SUMMARY: Thrombocytopenia is common in APS, and is likely associated with more severe disease. Improved understanding of thrombocytopenia in APS has the potential to improve risk stratification, reveal novel aspects of APS pathophysiology, and lead to treatments that are more individualized and holistic. Lippincott Williams And Wilkins 2019-05 2019-03-01 /pmc/articles/PMC6455093/ /pubmed/30747734 http://dx.doi.org/10.1097/BOR.0000000000000595 Text en Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. http://creativecommons.org/licenses/by-nc-nd/4.0 This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0
spellingShingle CLINICAL THERAPEUTICS & HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune
Vreede, Andrew P.
Bockenstedt, Paula L.
McCune, W. Joseph
Knight, Jason S.
Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title_full Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title_fullStr Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title_full_unstemmed Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title_short Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
title_sort cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome
topic CLINICAL THERAPEUTICS & HEMATOLOGIC COMPLICATIONS: Edited by W. Joseph McCune
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6455093/
https://www.ncbi.nlm.nih.gov/pubmed/30747734
http://dx.doi.org/10.1097/BOR.0000000000000595
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