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When specialties intersect: Acute coronary syndrome as the first clinical manifestation of myeloproliferative neoplasms
Quando as especialidades se cruzam: síndrome coronária aguda como a primeira manifestação clínica de neoplasias mieloproliferativas
Júlia Toste
Cardiovascular Centre, Hospital da Luz Lisboa, Lisbon, Portugal
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Uma série de casos" ] ] "contieneResumen" => array:2 [ "en" => true "pt" => true ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 666 "Ancho" => 700 "Tamanyo" => 29277 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">Case 4: coronary angiography image showing multiple thrombotic lesions in the left anterior descending artery at the time of anterior myocardial infarction.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "Betul Cengiz, Vedat Aytekin, Ulas Bildirici, Sukru Taylan Sahin, Selen Yurdakul, Saide Aytekin, Reyhan Kucukkaya" "autores" => array:7 [ 0 => array:2 [ "nombre" => "Betul" "apellidos" => "Cengiz" ] 1 => array:2 [ "nombre" => "Vedat" "apellidos" => "Aytekin" ] 2 => array:2 [ "nombre" => "Ulas" "apellidos" => "Bildirici" ] 3 => array:2 [ "nombre" => "Sukru Taylan" "apellidos" => "Sahin" ] 4 => array:2 [ "nombre" => "Selen" "apellidos" => "Yurdakul" ] 5 => array:2 [ "nombre" => "Saide" "apellidos" => "Aytekin" ] 6 => array:2 [ "nombre" => "Reyhan" "apellidos" => "Kucukkaya" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S2174204919302740" "doi" => "10.1016/j.repce.2019.12.003" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2174204919302740?idApp=UINPBA00004E" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0870255119305256?idApp=UINPBA00004E" "url" => "/08702551/0000003800000009/v2_202001221225/S0870255119305256/v2_202001221225/en/main.assets" ] "asociados" => array:1 [ 0 => array:20 [ "pii" => "S0870255119305256" "issn" => "08702551" "doi" => "10.1016/j.repc.2018.09.014" "estado" => "S300" "fechaPublicacion" => "2019-09-01" "aid" => "1441" "copyright" => "Sociedade Portuguesa de Cardiologia" "documento" => "article" "crossmark" => 1 "licencia" => "http://creativecommons.org/licenses/by-nc-nd/4.0/" "subdocumento" => "fla" "cita" => "Rev Port Cardiol. 2019;38:613-7" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 382 "formatos" => array:3 [ "EPUB" => 38 "HTML" => 229 "PDF" => 115 ] ] "en" => array:13 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Original Article</span>" "titulo" => "A rare cause of acute coronary syndromes in young adults – myeloproliferative neoplasms: A case series" "tienePdf" => "en" "tieneTextoCompleto" => "en" "tieneResumen" => array:2 [ 0 => "en" 1 => "pt" ] "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "613" "paginaFinal" => "617" ] ] "titulosAlternativos" => array:1 [ "pt" => array:1 [ "titulo" => "Uma causa rara de síndromes coronárias agudas em adultos jovens: neoplasias mieloproliferativas. 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All of the available data must be considered if the patient is to be properly diagnosed and treated.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Since cardiology focuses on cardiovascular diseases, cardiologists must bear in mind that what they encounter is often a complication of a more general disease. The article by Cengiz et al. published in this issue of the <span class="elsevierStyleItalic">Journal</span><a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">1</span></a> draws attention to this need, presenting a small case series of patients with myeloproliferative neoplasms (MPNs), the first manifestation of which was myocardial infarction (MI). The article highlights the need for careful assessment of blood cell count abnormalities in these patients, since appropriate hematological therapy is able to reduce their risk of thrombotic events in general and of acute coronary syndrome (ACS) in particular.</p><p id="par0015" class="elsevierStylePara elsevierViewall">When MI occurs in a patient who does not have traditional risk factors, alternative etiologies should be investigated.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">2</span></a> These include hypercoagulable states, coronary vasospasm, coronary inflammation, anomalous coronary arteries, coronary dissection, and embolization.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Patients across all age groups with MPNs are reported to be at increased risk for arterial and venous thrombosis compared with matched controls, with the highest rates at and shortly after diagnosis.<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">4</span></a> MPNs are clonal disorders of hematopoiesis characterized by proliferation of one or more myeloid lines in bone marrow. The most important in clinical practice are chronic myeloid leukemia and other Philadelphia chromosome-negative chronic MPNs,<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">5</span></a> including the classic Philadelphia chromosome-negative chronic MPNs polycythemia vera (PV), essential thrombocytosis (ET) and primary myelofibrosis (PMF) in the early/prefibrotic or the overt fibrotic stage. Less frequent chronic MPNs include chronic neutrophilic leukemia, unspecified chronic eosinophilic leukemia, and unclassified chronic MPNs.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The <span class="elsevierStyleItalic">JAK2</span> V617F mutation is detected in 90-95% of patients with PV, 60% of patients with ET and 60% of patients with PMF. The presence of <span class="elsevierStyleItalic">JAK2</span> mutations was included as a major diagnostic criterion in these three entities in the World Health Organization classification in 2008 and in the revised version published in 2016.<a class="elsevierStyleCrossRef" href="#bib0105"><span class="elsevierStyleSup">5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">MPNs are associated with hypercoagulable states and thrombotic complications are more prevalent than bleeding complications.<a class="elsevierStyleCrossRef" href="#bib0110"><span class="elsevierStyleSup">6</span></a> Thrombotic occlusions occur more frequently in arteries than veins. Stroke is the most frequent presentation, followed by MI and peripheral arterial occlusion.<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">2,3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Previous research into the pathogenesis of ACS has shown the importance of platelet thrombus formation caused by vascular endothelial damage resulting from plaque rupture.<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">7</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleItalic">JAK2</span> V617F-positive chronic MPNs commonly display dysfunction of integrins and adhesion molecules expressed on platelets, erythrocytes, and leukocytes.<a class="elsevierStyleCrossRef" href="#bib0120"><span class="elsevierStyleSup">8</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">Venous and arterial thrombosis is the major cause of morbidity and mortality in PV and ET patients.<a class="elsevierStyleCrossRefs" href="#bib0125"><span class="elsevierStyleSup">9,10</span></a> Strikingly, the thrombotic risk in these patients is dramatically higher than in patients with secondary erythrocytosis or thrombocytosis. This indicates that disease-intrinsic factors play a major role. Indeed, it has been reported that the <span class="elsevierStyleItalic">JAK2</span> V617F mutation leads to abnormal function of erythrocytes and platelets.<a class="elsevierStyleCrossRefs" href="#bib0135"><span class="elsevierStyleSup">11,12</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">PV generally causes increased production of granulocytes, platelets and erythrocytes, particularly the latter, which leads to hyperviscosity and increased risk of thrombosis.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">13</span></a> MI and sudden death are complications of newly diagnosed or untreated PV; they occur most often in elderly people (aged ≥65 years) with underlying coronary artery disease (CAD).<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">13</span></a> However, younger patients with PV who are free from CAD can also be affected, and sometimes the outcome is death.<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">13</span></a> In patients with PV, the main causes of mortality are MI and heart failure.<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">14</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Cytoreductive treatment of blood hyperviscosity by phlebotomy or chemotherapy and antiplatelet therapy with low-dose aspirin have dramatically reduced the incidence of thrombotic complications and substantially improved survival in PV.<a class="elsevierStyleCrossRef" href="#bib0155"><span class="elsevierStyleSup">15</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">In ACS patients with ET but without a previous history, it is not easy to recognize the underlying disease, but it is essential.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">16</span></a> Patients suffering ACS due to ET have few or no common risk factors for atherosclerosis, unlike other ACS cases. This is because the events are mainly due to thrombosis, as shown by lower plaque burden on angiography.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">16</span></a> Most cardiologists tend to focus more on the diagnosis and treatment of CAD, especially revascularization, than on blood cell count abnormalities, resulting in a missed diagnosis of ET in patients without marked thrombocytosis. Treatment of ACS with underlying ET should consist of three steps: cytoreduction, antithrombotic therapy and revascularization.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">16</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Combining their experience with other authors’ opinions, Xiong et al.<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">16</span></a> suggest the following triad as a red flag to look for ET in ACS:<ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">(1)</span><p id="par0070" class="elsevierStylePara elsevierViewall">ACS with no or few CAD-related risk factors;</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">(2)</span><p id="par0075" class="elsevierStylePara elsevierViewall">platelet count >450×10<span class="elsevierStyleSup">9</span>/l, even if only slightly above this level;</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">(3)</span><p id="par0080" class="elsevierStylePara elsevierViewall">no severe atherosclerotic narrowing found on coronary angiography, although thrombotic occlusion may be found.</p></li></ul></p><p id="par0085" class="elsevierStylePara elsevierViewall">Management of ET consists of antiplatelet therapy, mainly aspirin, and cytoreductive therapy that may include phlebotomy, hydroxyurea, anagrelide, interferon, and ruxolitinib, which is an inhibitor of JAK1 and JAK2.<a class="elsevierStyleCrossRef" href="#bib0095"><span class="elsevierStyleSup">3</span></a> Aspirin combined with cytoreductive therapy is considered to be highly effective in PV and ET patients.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">2</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">ACS occurring as the first manifestation of MPN is usually described in case reports. In their article, Cengiz et al.<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">1</span></a> present a small case series that draws attention to the need to be alert to the possibility of MPNs in young adults presenting with ACS, especially in the absence of atherosclerotic coronary artery lesions. It also stresses the importance of blood cell count abnormalities and the need for early diagnosis and initiation of specific treatment, which is essential to reduce the risk of further events and of mortality and morbidity related to thrombotic complications.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of interest</span><p id="par0095" class="elsevierStylePara elsevierViewall">The author has no conflicts of interest to declare.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:16 [ 0 => array:3 [ "identificador" => "bib0085" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A rare cause of acute coronary syndromes in young adults – myeloproliferative neoplasms: a case series" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:3 [ 0 => "B. 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| Ano/Mês | Html | Total | |
|---|---|---|---|
| 2024 Novembro | 7 | 12 | 19 |
| 2024 Outubro | 35 | 29 | 64 |
| 2024 Setembro | 44 | 18 | 62 |
| 2024 Agosto | 55 | 31 | 86 |
| 2024 Julho | 33 | 32 | 65 |
| 2024 Junho | 31 | 28 | 59 |
| 2024 Maio | 34 | 25 | 59 |
| 2024 Abril | 37 | 24 | 61 |
| 2024 Maro | 35 | 31 | 66 |
| 2024 Fevereiro | 48 | 44 | 92 |
| 2024 Janeiro | 29 | 47 | 76 |
| 2023 Dezembro | 26 | 38 | 64 |
| 2023 Novembro | 32 | 25 | 57 |
| 2023 Outubro | 19 | 18 | 37 |
| 2023 Setembro | 22 | 20 | 42 |
| 2023 Agosto | 22 | 21 | 43 |
| 2023 Julho | 47 | 12 | 59 |
| 2023 Junho | 34 | 11 | 45 |
| 2023 Maio | 31 | 27 | 58 |
| 2023 Abril | 18 | 11 | 29 |
| 2023 Maro | 36 | 20 | 56 |
| 2023 Fevereiro | 40 | 19 | 59 |
| 2023 Janeiro | 19 | 15 | 34 |
| 2022 Dezembro | 37 | 21 | 58 |
| 2022 Novembro | 49 | 31 | 80 |
| 2022 Outubro | 43 | 28 | 71 |
| 2022 Setembro | 22 | 29 | 51 |
| 2022 Agosto | 31 | 37 | 68 |
| 2022 Julho | 22 | 42 | 64 |
| 2022 Junho | 19 | 24 | 43 |
| 2022 Maio | 21 | 31 | 52 |
| 2022 Abril | 23 | 27 | 50 |
| 2022 Maro | 26 | 45 | 71 |
| 2022 Fevereiro | 22 | 60 | 82 |
| 2022 Janeiro | 13 | 27 | 40 |
| 2021 Dezembro | 15 | 30 | 45 |
| 2021 Novembro | 20 | 33 | 53 |
| 2021 Outubro | 27 | 44 | 71 |
| 2021 Setembro | 22 | 23 | 45 |
| 2021 Agosto | 26 | 22 | 48 |
| 2021 Julho | 18 | 27 | 45 |
| 2021 Junho | 22 | 19 | 41 |
| 2021 Maio | 21 | 26 | 47 |
| 2021 Abril | 31 | 37 | 68 |
| 2021 Maro | 43 | 13 | 56 |
| 2021 Fevereiro | 29 | 14 | 43 |
| 2021 Janeiro | 40 | 10 | 50 |
| 2020 Dezembro | 16 | 7 | 23 |
| 2020 Novembro | 22 | 27 | 49 |
| 2020 Outubro | 26 | 13 | 39 |
| 2020 Setembro | 21 | 21 | 42 |
| 2020 Agosto | 22 | 10 | 32 |
| 2020 Julho | 20 | 10 | 30 |
| 2020 Junho | 15 | 13 | 28 |
| 2020 Maio | 19 | 5 | 24 |
| 2020 Abril | 16 | 12 | 28 |
| 2020 Maro | 27 | 14 | 41 |
| 2020 Fevereiro | 35 | 31 | 66 |
| 2020 Janeiro | 129 | 45 | 174 |
| 2019 Dezembro | 26 | 17 | 43 |
| 2019 Novembro | 10 | 5 | 15 |
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