Letter to the Editor
Pulmonary thrombotic microangiopathy caused by neoplastic urothelial carcinoma cells
Microangiopatía trombótica pulmonar por células neoplásicas de cáncer urotelial
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B) CKAE1-AE3 immunohistochemical technique: microscopic image of the lung parenchyma, cross section of an artery, reveals a group of positive tumour cells in its lumen (red arrow). The colour of the image is only visible in the electronic version.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Tumour thrombotic microangiopathy is a rare clinicopathological entity related to malignant neoplasms, difficult to diagnose ante mortem. It is characterised by pulmonary complications with rapid progression of dyspnoea and pulmonary hypertension leading to a poor prognosis.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> We report the case of a pulmonary tumour thrombotic microangiopathy (PTTM) due to urothelial cancer.</p><p id="par0010" class="elsevierStylePara elsevierViewall">This is a 57-year-old man with no medical history who came to the emergency department for chest pain and dyspnoea. The electrocardiogram showed an S1Q3T3 pattern and the blood test showed elevated <span class="elsevierStyleSmallCaps">d</span>-dimer levels (14,000<span class="elsevierStyleHsp" style=""></span>ng feu/mL). Computed tomography was negative for pulmonary embolism (PE), and echocardiography showed signs of right ventricular overload. Given a high suspicion of PE, anticoagulation treatment was started. Doppler imaging of the lower limbs ruled out thrombosis in the venous territory and the study was completed with a ventilation-perfusion scintigraphy that showed repletion defects in subsegmental vessels, suggestive but not conclusive of PE. After 72<span class="elsevierStyleHsp" style=""></span>h, thrombocytopenia (13,000<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleSup">3</span>), anaemia (Hb 10.9<span class="elsevierStyleHsp" style=""></span>g/dl), elevated total bilirubin (3.34<span class="elsevierStyleHsp" style=""></span>mg/dl) and LDH (719<span class="elsevierStyleHsp" style=""></span>U/l) were evident, and low haptoglobin (<2.56<span class="elsevierStyleHsp" style=""></span>mg/dl, reference values 32−197<span class="elsevierStyleHsp" style=""></span>mg/dl), so anticoagulation was discontinued due to risk of haemorrhage. Given the suspicion of thrombotic microangiopathy, a peripheral blood smear was performed which showed schistocytes, concordant with the clinical suspicion of thrombotic thrombocytopenic purpura, ADAMST13 was requested and the patient was admitted to the intensive care unit to start plasmapheresis and respiratory support. Eight hours after admission to the intensive care unit, emergency intubation was performed due to increased work of breathing, with subsequent cardiorespiratory arrest and unsuccessful cardiopulmonary resuscitation manoeuvres, resulting in death.</p><p id="par0015" class="elsevierStylePara elsevierViewall">An autopsy was requested which revealed microvascular pulmonary tumour embolism with images of tumour thrombi in medium calibre arteries of both lungs, metastasis of micropapillary carcinoma with immunophenotype consistent with urothelial origin (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>) and dilatation of right cavities secondary to underlying disease (metastatic tumour PE).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">PTTM is a rare clinicopathological entity characterised by microscopic tumour embolization and fibrocellular proliferation of the intima of small arterioles, first described in 1990 by von Hervay et al. The most common origin is stomach cancer, followed by lung, breast, pancreatic and oesophageal cancer.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The most common symptoms are progressive dyspnoea, cough, and haemoptysis, attributed to pulmonary arterial hypertension and right heart failure, leading to a misdiagnosis of idiopathic pulmonary arterial hypertension.</p><p id="par0030" class="elsevierStylePara elsevierViewall">Pathological findings in the biopsy are essential for diagnostic confirmation. The radiological diagnosis has not been established because the findings are usually minimal or non-specific.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">This case shows the complexity of PTTM diagnosis and its aggressive course once symptoms appear. Ante mortem diagnosis and treatment is essential before the patient develops pulmonary hypertension, so it is important to suspect microangiopathy, pulmonary hypertension, absence of macroscopic pulmonary emboli and sub-segmental defects on perfusion scintigraphy.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:1 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1063 "Ancho" => 2007 "Tamanyo" => 456398 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A) H&E technique: microscopic image of lung parenchyma showing the alveoli and, on the right, a longitudinal section of an artery with the presence of a cluster of tumour cells in its lumen (red arrow). B) CKAE1-AE3 immunohistochemical technique: microscopic image of the lung parenchyma, cross section of an artery, reveals a group of positive tumour cells in its lumen (red arrow). The colour of the image is only visible in the electronic version.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:4 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Pulmonary tumor thrombotic microangiopathy: a systematic review" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "R.H. Godbole" 1 => "R. Saggar" 2 => "N. Kamangar" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1177/2045894019851000" "Revista" => array:5 [ "tituloSerie" => "Pulm Circ." 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"fecha" => "2018" "volumen" => "8" "paginaInicial" => "127" "paginaFinal" => "131" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/32995214" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/23870206/0000016000000005/v1_202303052139/S2387020623000554/v1_202303052139/en/main.assets" "Apartado" => array:4 [ "identificador" => "43309" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Letters to the Editor" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/23870206/0000016000000005/v1_202303052139/S2387020623000554/v1_202303052139/en/main.pdf?idApp=UINPBA00004N&text.app=https://www.elsevier.es/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S2387020623000554?idApp=UINPBA00004N" ]
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