Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension
Pulmonary arterial hypertension is a rare disease of the pulmonary vasculature, characterised pathologically by proliferation, remodelling and thrombosis in situ . Unfortunately, existing therapeutic interventions do not reverse these findings and the disease continues to result in significant morbi...
| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Wiley
2021-06-01
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| Series: | Pulmonary Circulation |
| Online Access: | https://doi.org/10.1177/20458940211021036 |
| _version_ | 1811319395122675712 |
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| author | Sarah Cullivan Claire A. Murphy Luisa Weiss Shane P. Comer Barry Kevane Brian McCullagh Patricia B. Maguire Fionnuala Ní Ainle Sean P. Gaine |
| author_facet | Sarah Cullivan Claire A. Murphy Luisa Weiss Shane P. Comer Barry Kevane Brian McCullagh Patricia B. Maguire Fionnuala Ní Ainle Sean P. Gaine |
| author_sort | Sarah Cullivan |
| collection | DOAJ |
| description | Pulmonary arterial hypertension is a rare disease of the pulmonary vasculature, characterised pathologically by proliferation, remodelling and thrombosis in situ . Unfortunately, existing therapeutic interventions do not reverse these findings and the disease continues to result in significant morbidity and premature mortality. A number of haematological derangements have been described in pulmonary arterial hypertension which may provide insights into the pathobiology of the disease and opportunities to explore new therapeutic pathways. These include quantitative and qualitative platelet abnormalities, such as thrombocytopaenia, increased mean platelet volume and altered platelet bioenergetics. Furthermore, a hypercoagulable state and aberrant negative regulatory pathways can be observed, which could contribute to thrombosis in situ in distal pulmonary arteries and arterioles. Finally, there is increasing interest in the role of extracellular vesicle autocrine and paracrine signalling in pulmonary arterial hypertension, and their potential utility as biomarkers and novel therapeutic targets. This review focuses on the potential role of platelets, extracellular vesicles and coagulation pathways in the pathobiology of pulmonary arterial hypertension. We highlight important unanswered clinical questions and the implications of these observations for future research and pulmonary arterial hypertension-directed therapies. |
| first_indexed | 2024-04-13T12:40:47Z |
| format | Article |
| id | doaj.art-3123a8955da5418ba1b4d6a5adc91a19 |
| institution | Directory Open Access Journal |
| issn | 2045-8940 |
| language | English |
| last_indexed | 2024-04-13T12:40:47Z |
| publishDate | 2021-06-01 |
| publisher | Wiley |
| record_format | Article |
| series | Pulmonary Circulation |
| spelling | doaj.art-3123a8955da5418ba1b4d6a5adc91a192022-12-22T02:46:30ZengWileyPulmonary Circulation2045-89402021-06-011110.1177/20458940211021036Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertensionSarah CullivanClaire A. MurphyLuisa WeissShane P. ComerBarry KevaneBrian McCullaghPatricia B. MaguireFionnuala Ní AinleSean P. GainePulmonary arterial hypertension is a rare disease of the pulmonary vasculature, characterised pathologically by proliferation, remodelling and thrombosis in situ . Unfortunately, existing therapeutic interventions do not reverse these findings and the disease continues to result in significant morbidity and premature mortality. A number of haematological derangements have been described in pulmonary arterial hypertension which may provide insights into the pathobiology of the disease and opportunities to explore new therapeutic pathways. These include quantitative and qualitative platelet abnormalities, such as thrombocytopaenia, increased mean platelet volume and altered platelet bioenergetics. Furthermore, a hypercoagulable state and aberrant negative regulatory pathways can be observed, which could contribute to thrombosis in situ in distal pulmonary arteries and arterioles. Finally, there is increasing interest in the role of extracellular vesicle autocrine and paracrine signalling in pulmonary arterial hypertension, and their potential utility as biomarkers and novel therapeutic targets. This review focuses on the potential role of platelets, extracellular vesicles and coagulation pathways in the pathobiology of pulmonary arterial hypertension. We highlight important unanswered clinical questions and the implications of these observations for future research and pulmonary arterial hypertension-directed therapies.https://doi.org/10.1177/20458940211021036 |
| spellingShingle | Sarah Cullivan Claire A. Murphy Luisa Weiss Shane P. Comer Barry Kevane Brian McCullagh Patricia B. Maguire Fionnuala Ní Ainle Sean P. Gaine Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension Pulmonary Circulation |
| title | Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension |
| title_full | Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension |
| title_fullStr | Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension |
| title_full_unstemmed | Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension |
| title_short | Platelets, extracellular vesicles and coagulation in pulmonary arterial hypertension |
| title_sort | platelets extracellular vesicles and coagulation in pulmonary arterial hypertension |
| url | https://doi.org/10.1177/20458940211021036 |
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