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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; PET axial image of the chest revealing hypermetabolic circumferential thickening of the pleural surface of the right hemithorax&#46; Note the increased thickening of the posterior pleural surface &#40;arrows&#41;&#46; &#40;B&#41; PET coronal image revealing hypermetabolic thickening of the pleural surface of the right hemithorax &#40;short arrows&#41;&#46; Note also the intense FDG uptake by the oblique fissure &#40;long arrow&#41;&#46; &#40;C&#41; PET&#8211;CT fusion axial image showing marked hypermetabolic thickening of the pleural surface of the right hemithorax at the level of the posterior costophrenic angle&#46; On the basis of these images&#44; this region was selected for biopsy&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Richter syndrome &#40;RS&#41; consists of the transformation of chronic lymphocytic leukemia &#40;CLL&#41; to diffuse large B-cell lymphoma &#40;DLBCL&#41;&#44; a rapidly growing variety of non-Hodgkin&#39;s lymphoma with poor prognosis&#46; The disease course in 5&#37;&#8211;10&#37; of patients with CLL is complicated by RS&#44; which generally presents clinically in the form of lymphadenopathies&#44; splenomegaly&#44; and worsening B symptoms &#40;fever&#44; night sweats&#44; weight loss&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Positron emission tomography-computed tomography &#40;PET&#8211;CT&#41; is a powerful hybrid diagnostic tool that is useful in patients with RS because it helps plan and obtain histological biopsies of lesions with greater metabolism using other techniques&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 74-year-old woman diagnosed with CLL in 2009&#44; who attended the emergency department of our hospital with a 2-week history of progressive dyspnea&#44; chest discomfort and low-grade fever&#46; Chest radiograph revealed a large right pleural effusion causing mediastinal shift to the contralateral side&#44; and secondary atelectasis of a large part of the right lung&#46; A pleural tube was placed in the emergency room&#44; draining abundant serosanguineous fluid&#46; A whole-body PET&#8211;CT scan with intravenous contrast and F18-fluordeoxyglucose &#40;FDG&#41; performed 3 days later showed marked diffuse hypermetabolic thickening of the pleural surface of the right hemithorax&#44; with secondary compressive atelectasis of the ipsilateral lung&#44; which did not show pathological FDG uptake &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; No lymphadenopathies&#44; signs of bone marrow infiltration&#44; or other hypermetabolic lesions were observed in the abdominal viscera or the skeleton&#46; In view of these findings&#44; a core needle biopsy was performed in the thickest area of the pleura with the highest metabolism on PET&#8211;CT&#44; confirming the diagnosis of an aggressive DLBCL&#46; The patient initially improved with pleural drainage and chemotherapy &#40;rituximab&#44; cyclophosphamide&#44; doxorubicin&#44; vincristine&#44; and prednisone&#41;&#44; but died 4 months later due to systemic progressive disease&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Most RS present with nodal &#40;lymph nodes&#41; and bone marrow involvement&#44; although atypical forms of extranodal involvement have been described in the digestive tract&#44; the pulmonary parenchyma and the skin&#46; Our case is exceptional because we could not find any reports in the literature of RS presenting with exclusively pleural extranodal involvement&#59; we only found some isolated references to RS occurring with pleural effusion&#44; but in general&#44; it is always associated with the presence of lymphadenopathies in other sites&#44; with no diffuse thickening of the pleural surface&#46; Our patient&#44; then&#44; is a case of DLBCL with exclusively pleural extranodal involvement complicating the course of her CLL&#46; Two forms of primary pleural lymphoma are described in the literature&#58; body cavity lymphoma &#40;lymphomatous pleural effusion&#41;&#44; which usually affects patients with acquired immunodeficiency syndrome and is caused by the human herpes virus type 8&#59; and lymphoma associated with pyothorax&#44; recently renamed DLBCL associated with chronic inflammation&#44; which often affects patients with fibrothorax caused by tuberculosis&#44; although it has also been described in association with chronic osteomyelitis of the chest wall&#44; thoracoplasty&#44; or the presence of metal implants &#40;this form of primary pleural lymphoma has also been associated with Epstein&#8211;Barr virus infection&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Our patient did not belong to either of these 2 groups of primary pleural lymphoma&#46; In line with recent articles that highlight the importance of PET&#8211;CT in the diagnostic management of RS&#44; performing this imaging study in our patient &#40;as the only whole-body diagnostic imaging procedure&#41; was very useful for demonstrating the intense hypermetabolism of the pleural surface of the right hemithorax and for ruling out hypermetabolic lesions in other sites&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3&#44;5</span></a> The lack of FDG uptake in the right pulmonary parenchyma&#44; which was confirmed by the CT component of the PET&#8211;CT&#44; prompted us to obtain a radiological-guided percutaneous core needle biopsy in the area of hypermetabolic pleural thickening&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Our case illustrates an unusual form of extranodal&#44; exclusively pleural RS in a patient with a history of CLL&#46; It also illustrates the great potential of the PET&#8211;CT hybrid technique in the diagnostic management of RS as a guide for obtaining histological samples of lesions with increased metabolic activity&#44; 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Scientific Letter
Richter Syndrome With Extensive Isolated Pleural Extranodal Involvement: The Importance of PET/CT Imaging
Síndrome de Richter con extensa afectación extranodal pleural aislada: importancia de la PET/TC
Luis Gorospe Sarasúaa,
Corresponding author
luisgorospe@yahoo.com

Corresponding author.
, Ana Jaureguízar-Oriolb, Carlos Almonacid-Sánchezb, María Eugenia Rioja-Martínc
a Servicio de Radiodiagnóstico, Hospital Universitario Ramón y Cajal, Madrid, Spain
b Servicio de Neumología, Hospital Universitario Ramón y Cajal, Madrid, Spain
c Servicio de Medicina Nuclear, Hospital Universitario Ramón y Cajal, Madrid, Spain
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        "titulo" => "S&#237;ndrome de Richter con extensa afectaci&#243;n extranodal pleural aislada&#58; importancia de la PET&#47;TC"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; PET axial image of the chest revealing hypermetabolic circumferential thickening of the pleural surface of the right hemithorax&#46; Note the increased thickening of the posterior pleural surface &#40;arrows&#41;&#46; &#40;B&#41; PET coronal image revealing hypermetabolic thickening of the pleural surface of the right hemithorax &#40;short arrows&#41;&#46; Note also the intense FDG uptake by the oblique fissure &#40;long arrow&#41;&#46; &#40;C&#41; PET&#8211;CT fusion axial image showing marked hypermetabolic thickening of the pleural surface of the right hemithorax at the level of the posterior costophrenic angle&#46; On the basis of these images&#44; this region was selected for biopsy&#46;</p>"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Richter syndrome &#40;RS&#41; consists of the transformation of chronic lymphocytic leukemia &#40;CLL&#41; to diffuse large B-cell lymphoma &#40;DLBCL&#41;&#44; a rapidly growing variety of non-Hodgkin&#39;s lymphoma with poor prognosis&#46; The disease course in 5&#37;&#8211;10&#37; of patients with CLL is complicated by RS&#44; which generally presents clinically in the form of lymphadenopathies&#44; splenomegaly&#44; and worsening B symptoms &#40;fever&#44; night sweats&#44; weight loss&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Positron emission tomography-computed tomography &#40;PET&#8211;CT&#41; is a powerful hybrid diagnostic tool that is useful in patients with RS because it helps plan and obtain histological biopsies of lesions with greater metabolism using other techniques&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 74-year-old woman diagnosed with CLL in 2009&#44; who attended the emergency department of our hospital with a 2-week history of progressive dyspnea&#44; chest discomfort and low-grade fever&#46; Chest radiograph revealed a large right pleural effusion causing mediastinal shift to the contralateral side&#44; and secondary atelectasis of a large part of the right lung&#46; A pleural tube was placed in the emergency room&#44; draining abundant serosanguineous fluid&#46; A whole-body PET&#8211;CT scan with intravenous contrast and F18-fluordeoxyglucose &#40;FDG&#41; performed 3 days later showed marked diffuse hypermetabolic thickening of the pleural surface of the right hemithorax&#44; with secondary compressive atelectasis of the ipsilateral lung&#44; which did not show pathological FDG uptake &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; No lymphadenopathies&#44; signs of bone marrow infiltration&#44; or other hypermetabolic lesions were observed in the abdominal viscera or the skeleton&#46; In view of these findings&#44; a core needle biopsy was performed in the thickest area of the pleura with the highest metabolism on PET&#8211;CT&#44; confirming the diagnosis of an aggressive DLBCL&#46; The patient initially improved with pleural drainage and chemotherapy &#40;rituximab&#44; cyclophosphamide&#44; doxorubicin&#44; vincristine&#44; and prednisone&#41;&#44; but died 4 months later due to systemic progressive disease&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Most RS present with nodal &#40;lymph nodes&#41; and bone marrow involvement&#44; although atypical forms of extranodal involvement have been described in the digestive tract&#44; the pulmonary parenchyma and the skin&#46; Our case is exceptional because we could not find any reports in the literature of RS presenting with exclusively pleural extranodal involvement&#59; we only found some isolated references to RS occurring with pleural effusion&#44; but in general&#44; it is always associated with the presence of lymphadenopathies in other sites&#44; with no diffuse thickening of the pleural surface&#46; Our patient&#44; then&#44; is a case of DLBCL with exclusively pleural extranodal involvement complicating the course of her CLL&#46; Two forms of primary pleural lymphoma are described in the literature&#58; body cavity lymphoma &#40;lymphomatous pleural effusion&#41;&#44; which usually affects patients with acquired immunodeficiency syndrome and is caused by the human herpes virus type 8&#59; and lymphoma associated with pyothorax&#44; recently renamed DLBCL associated with chronic inflammation&#44; which often affects patients with fibrothorax caused by tuberculosis&#44; although it has also been described in association with chronic osteomyelitis of the chest wall&#44; thoracoplasty&#44; or the presence of metal implants &#40;this form of primary pleural lymphoma has also been associated with Epstein&#8211;Barr virus infection&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Our patient did not belong to either of these 2 groups of primary pleural lymphoma&#46; In line with recent articles that highlight the importance of PET&#8211;CT in the diagnostic management of RS&#44; performing this imaging study in our patient &#40;as the only whole-body diagnostic imaging procedure&#41; was very useful for demonstrating the intense hypermetabolism of the pleural surface of the right hemithorax and for ruling out hypermetabolic lesions in other sites&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">2&#44;3&#44;5</span></a> The lack of FDG uptake in the right pulmonary parenchyma&#44; which was confirmed by the CT component of the PET&#8211;CT&#44; prompted us to obtain a radiological-guided percutaneous core needle biopsy in the area of hypermetabolic pleural thickening&#46;</p><p id="par0020" class="elsevierStylePara elsevierViewall">Our case illustrates an unusual form of extranodal&#44; exclusively pleural RS in a patient with a history of CLL&#46; It also illustrates the great potential of the PET&#8211;CT hybrid technique in the diagnostic management of RS as a guide for obtaining histological samples of lesions with increased metabolic activity&#44; thus increasing the diagnostic yield of the biopsy&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Gorospe Saras&#250;a L&#44; Jauregu&#237;zar-Oriol A&#44; Almonacid-S&#225;nchez C&#44; Rioja-Mart&#237;n ME&#46; S&#237;ndrome de Richter con extensa afectaci&#243;n extranodal pleural aislada&#58; importancia de la PET&#47;TC&#46; Arch Bronconeumol&#46; 2017&#59;53&#58;644&#8211;646&#46;</p>"
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          "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">&#40;A&#41; PET axial image of the chest revealing hypermetabolic circumferential thickening of the pleural surface of the right hemithorax&#46; Note the increased thickening of the posterior pleural surface &#40;arrows&#41;&#46; &#40;B&#41; PET coronal image revealing hypermetabolic thickening of the pleural surface of the right hemithorax &#40;short arrows&#41;&#46; Note also the intense FDG uptake by the oblique fissure &#40;long arrow&#41;&#46; &#40;C&#41; PET&#8211;CT fusion axial image showing marked hypermetabolic thickening of the pleural surface of the right hemithorax at the level of the posterior costophrenic angle&#46; On the basis of these images&#44; this region was selected for biopsy&#46;</p>"
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Article information
ISSN: 15792129
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