The prothrombotic state in paroxysmal nocturnal hemoglobinuria: a multifaceted source

Peacock-Young, Barnaby; Macrae, Fraser L.; Newton, Darren J.; Hill, Anita; Ariëns, Robert A. S.

doi:10.3324/haematol.2017.177618

Cited by 34 publications

(20 citation statements)

References 122 publications

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“…Indeed, free hemoglobin released during hemolysis binds to haptoglobin that will subsequently bind to its receptor (CD163) on activated macrophages [ 23 ]. As shown in hereditary or acquired hemolytic anemias [ 10 , 24 , 25 ], once this mechanism is saturated due to high hemolysis rate, the increase in free hemoglobin in the plasma scavenges NO, thus resulting in a prothrombotic state, due to vasoconstriction, activation of endothelial cells and platelet aggregation. However, none of these parameters were significantly different between the two groups ( Fig 2 ).…”

Section: Resultsmentioning

confidence: 99%

Venous thromboembolic events during warm autoimmune hemolytic anemia

Audia¹,

Bach²,

Samson³

et al. 2018

PLoS ONE

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Thrombotic manifestations are a hallmark of many auto-immune diseases (AID), specially of warm autoimmune hemolytic anemia (wAIHA), as 15 to 33% of adults with wAIHA experience venous thromboembolic events (VTE). However, beyond the presence of positive antiphospholipid antibodies and splenectomy, risk factors for developing a VTE during wAIHA have not been clearly identified. The aim of this retrospective study was to characterize VTEs during wAIHA and to identify risk factors for VTE. Forty-eight patients with wAIHA were included, among whom 26 (54%) had secondary wAIHA. Eleven (23%) patients presented at least one VTE, that occurred during an active phase of the disease for 10/11 patients (90%). The frequency of VTE was not different between primary and secondary AIHA (23.7 vs. 19.2%; p = 0.5). The Padua prediction score based on traditional risk factors was not different between patients with and without VTE. On multivariate analysis, total bilirubin ≥ 40 μmol/L [odds ratio (OR) = 7.4; p = 0.02] and leucocyte count above 7x109/L (OR = 15.7; p = 0.02) were significantly associated with a higher risk of thrombosis. Antiphospholipid antibodies were screened in 9 out the 11 patients who presented a VTE and were negative. Thus, the frequency of VTE is high (23%) during wAIHA and VTE preferentially occur within the first weeks of diagnosis. As no clinically relevant predictive factors of VTE could be identified, the systematic use of a prophylactic anticoagulation should be recommended in case of active hemolysis and its maintenance after hospital discharge should be considered. The benefit of a systematic screening for VTE and its procedure remain to be determined.

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Section: Resultsmentioning

confidence: 99%

Venous thromboembolic events during warm autoimmune hemolytic anemia

Audia¹,

Bach²,

Samson³

et al. 2018

PLoS ONE

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“…). Massive hemolysis followed by thrombotic complications is a major pathogenic mechanism in paroxysmal nocturnal hemoglobinuria and in adverse effects of RBC transfusions, provided they are not caused by the underlying disease . Hemolysis is accompanied by (pro)thrombotic conditions that can range from mild hypercoagulability detected by laboratory signs to life‐threatening complications, such as disseminated intravascular coagulation and venous thromboembolism .…”

Section: Quantitative and Qualitative Changes In Rbcs Related To Bleementioning

confidence: 99%

Red blood cells: the forgotten player in hemostasis and thrombosis

Weisel

Litvinov

2019

Journal of Thrombosis and Haemostasis

325

272

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Summary New evidence has stirred up a long‐standing but undeservedly forgotten interest in the role of erythrocytes, or red blood cells (RBCs), in blood clotting and its disorders. This review summarizes the most recent research that describes the involvement of RBCs in hemostasis and thrombosis. There are both quantitative and qualitative changes in RBCs that affect bleeding and thrombosis, as well as interactions of RBCs with cellular and molecular components of the hemostatic system. The changes in RBCs that affect hemostasis and thrombosis include RBC counts or hematocrit (modulating blood rheology through viscosity) and qualitative changes, such as deformability, aggregation, expression of adhesive proteins and phosphatidylserine, release of extracellular microvesicles, and hemolysis. The pathogenic mechanisms implicated in thrombotic and hemorrhagic risk include variable adherence of RBCs to the vessel wall, which depends on the functional state of RBCs and/or endothelium, modulation of platelet reactivity and platelet margination, alterations of fibrin structure and reduced susceptibility to fibrinolysis, modulation of nitric oxide availability, and the levels of von Willebrand factor and factor VIII in blood related to the ABO blood group system. RBCs are involved in platelet‐driven contraction of clots and thrombi that results in formation of a tightly packed array of polyhedral erythrocytes, or polyhedrocytes, which comprises a nearly impermeable barrier that is important for hemostasis and wound healing. The revisited notion of the importance of RBCs is largely based on clinical and experimental associations between RBCs and thrombosis or bleeding, implying that RBCs are a prospective therapeutic target in hemostatic and thrombotic disorders.

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“…Estima-se que até 40% dos pacientes acometidos irão manifestar trombose venosa em algum momento da vida e desses, uma parcela pode evoluir com tromboembolismo. Em geral, há predileção pelo plexo intra-abdominal, principalmente as veias renais e hepática, sendo a Síndrome de Budd-Chiari uma complicação grave (PEACOCK-YOUNG et al, 2018). Por esse motivo, institui-se a profilaxia com Varfarina para aqueles que não possuem contraindicações a uma anticoagulação crônica.…”

Section: Discussionunclassified

“…Por essa razão, pacientes com anemia hemolítica crônica adquirida e teste de coombs negativo devem ser investigados (ARAUJO et al, 2019). Após o diagnóstico é essencial a pesquisa de possíveis complicações relacionadas à patologia, como a trombose da veia hepática, mais conhecida como Síndrome de Budd-Chiari, uma das mais temidas e letais (PEACOCK-YOUNG et al, 2018).…”

Section: Conclusãounclassified

Panorama Do Trauma Durante O Nascimento No Brasil No Período De 2009 a 2018: Um Estudo Ecológico De Série Temporal

Carvalho¹,

Cerqueira²,

Alves³

et al. 2020

Saúde Da Criança E Do Adolescente: Instrumentos Norteadores E De Acompanhamento

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Todo o conteúdo deste livro está licenciado sob uma Licença de Atribuição Creative Commons. Atribuição 4.0 Internacional (CC BY 4.0). O conteúdo dos artigos e seus dados em sua forma, correção e confiabilidade são de responsabilidade exclusiva dos autores. Permitido o download da obra e o compartilhamento desde que sejam atribuídos créditos aos autores, mas sem a possibilidade de alterá-la de nenhuma forma ou utilizá-la para fins comerciais.

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The prothrombotic state in paroxysmal nocturnal hemoglobinuria: a multifaceted source

Cited by 34 publications

References 122 publications

Venous thromboembolic events during warm autoimmune hemolytic anemia

Venous thromboembolic events during warm autoimmune hemolytic anemia

Red blood cells: the forgotten player in hemostasis and thrombosis

Panorama Do Trauma Durante O Nascimento No Brasil No Período De 2009 a 2018: Um Estudo Ecológico De Série Temporal

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