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1</a>&#41;&#46; The plaque had appeared 3 weeks earlier&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy was performed to investigate the suspected diagnosis of primary cutaneous lymphoma&#44; with results showing a diffuse&#44; dense infiltration of large&#44; highly pleomorphic atypical basophilic cells in the dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immunohistochemical stains were positive for B-cell markers &#40;CD20&#44; CD79a&#41;&#44; negative for T-cell markers &#40;CD3&#44; CD5&#41;&#44; and strongly positive for Bcl-2&#46; Less intense staining was observed for Bcl-6 and MUM-1&#44; and the results were negative for CD10 and CD30 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A and B&#41;&#46; Ki-67 staining showed high proliferative activity &#40;90&#37;&#41;&#46; Fluorescent in situ hybridization was negative for <span class="elsevierStyleItalic">Bcl-2</span> rearrangement with the t&#40;14&#59;18&#41; translocation&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">A computed tomography staging study revealed multiple enlarged infradiaphragmatic and supradiaphragmatic lymph nodes and a tumor measuring 6<span class="elsevierStyleHsp" style=""></span>cm on the left kidney&#46; Bone marrow biopsy showed an infiltration of small cells with positive B-cell markers&#46; Unlike the skin biopsy&#44; the bone marrow biopsy showed CD5 expression and an absence of Bcl-6 and MUM-1 expression&#44; leading to a diagnosis of chronic lymphocytic leukemia&#46; The definitive diagnosis was thus systemic DLBCL with secondary cutaneous involvement due to a previous unknown transformation of chronic lymphocytic leukemia &#40;Richter syndrome or transformation&#41;&#46; The patient was treated with R-CHOP&#44; which initially resulted in a reduction in the size of the skin tumor and the lymph nodes&#46; However&#44; he died 4 months later due to neutropenic sepsis&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">DLBCL is a heterogeneous entity with multiple clinical and pathologic variants&#46; It is most common in individuals aged over 60 years&#46; It is also the most common type of lymphoma in patients with human immunodeficiency virus infection&#46; Because of its high proliferative activity&#44; it is sometimes diagnosed earlier than other more indolent lymphomas&#46; Immunophenotypically&#44; all DLBCLs express B-cell markers&#44; and other markers are expressed in a variable proportion of tumors &#40;Bcl-6 in 60&#37;-90&#37;&#44; MUM-1 in 35&#37;-65&#37;&#44; and Bcl-2 in 50&#37;&#41;&#46; Although Bcl-2 is expressed in over 60&#37; of tumors&#44; <span class="elsevierStyleItalic">Bcl-2</span> rearrangement is observed in under 20&#37; of cases&#44; suggesting the existence of other genetic pathways for the expression of this protein&#46; There has also been a description of a double-hit variant involving both <span class="elsevierStyleItalic">Bcl-2</span> and <span class="elsevierStyleItalic">Myc</span> rearrangement associated with worse prognosis and a greater tendency for secondary cutaneous involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The primary cutaneous variant of DLBCL&#44; primary cutaneous diffuse large B-cell lymphoma &#40;PCDLBCL&#41;&#44; leg type&#44; is one of the 3 most common types of primary cutaneous B-cell lymphomas&#44; together with primary cutaneous follicle center lymphoma and marginal zone B-cell lymphoma&#46; PCDLBCL&#44; leg-type presents as solitary or multiple violaceous plaques that typically occur on the legs of elderly patients&#46; It is by far the primary cutaneous B-cell lymphoma with the worst prognosis&#44; with a 5-year survival of 50&#37;&#46; It is therefore essential to initiate treatment with multi-agent chemotherapy and rituximab as early as possible&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Because the immunohistochemical markers are the same for PCDLBCL&#44; leg type and its systemic variant&#44; DLBCL&#44; according to the current WHO-EORTC criteria&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> definitive diagnosis should be based on the presence or absence of extracutaneous disease at diagnosis&#46; Testicular B-cell lymphoma can also affect the skin and its pathologic and immunophenotypic characteristics are indistinguishable from those of PCDLBCL&#44; leg type&#59; routine testicular examination is therefore recommended in appropriate cases&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">We have reported a case of systemic DLBCL diagnosed following the detection of a cutaneous lesion without other signs or symptoms&#46; One particularly interesting aspect of this case was the presence of a solitary lesion that clinically resembled a primary cutaneous lymphoma&#46;</p></span>"
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Case and Research Letters
Diffuse Systemic Large B-Cell Lymphoma With Secondary Skin Involvement
Linfoma B difuso de células grandes sistémico con afectación cutánea secundaria
C. Lloret-Ruiz
Corresponding author
clloretruiz@gmail.com

Corresponding author.
, P. Molés-Poveda, N. Barrado-Solís, E. Gimeno-Carpio
Servicio de Dermatología, Hospital Arnau de Vilanova, Valencia, Spain
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    "titulo" => "Diffuse Systemic Large B-Cell Lymphoma With Secondary Skin Involvement"
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        "titulo" => "Linfoma B difuso de c&#233;lulas grandes sist&#233;mico con afectaci&#243;n cut&#225;nea secundaria"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Diffuse large B-cell lymphoma &#40;DLBCL&#41; is the most common type of systemic lymphoma and accounts for 40&#37; of all new cases of this disease&#46; Prognosis has improved considerably since the introduction of rituximab&#44; which combined with chemotherapy is associated with a cure rate of up to 50&#37;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a>&#59; R-CHOP is the regimen of choice&#46; Secondary cutaneous involvement is uncommon in DLBCL&#44; although it has been observed in up to 20&#37; of cases in some series&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of an 81-year-old man in good health who presented with an indurated&#44; erythematous-violaceous plaque-like lesion with a diameter of 7<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm on the right leg &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; The plaque had appeared 3 weeks earlier&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy was performed to investigate the suspected diagnosis of primary cutaneous lymphoma&#44; with results showing a diffuse&#44; dense infiltration of large&#44; highly pleomorphic atypical basophilic cells in the dermis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immunohistochemical stains were positive for B-cell markers &#40;CD20&#44; CD79a&#41;&#44; negative for T-cell markers &#40;CD3&#44; CD5&#41;&#44; and strongly positive for Bcl-2&#46; Less intense staining was observed for Bcl-6 and MUM-1&#44; and the results were negative for CD10 and CD30 &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A and B&#41;&#46; Ki-67 staining showed high proliferative activity &#40;90&#37;&#41;&#46; Fluorescent in situ hybridization was negative for <span class="elsevierStyleItalic">Bcl-2</span> rearrangement with the t&#40;14&#59;18&#41; translocation&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">A computed tomography staging study revealed multiple enlarged infradiaphragmatic and supradiaphragmatic lymph nodes and a tumor measuring 6<span class="elsevierStyleHsp" style=""></span>cm on the left kidney&#46; Bone marrow biopsy showed an infiltration of small cells with positive B-cell markers&#46; Unlike the skin biopsy&#44; the bone marrow biopsy showed CD5 expression and an absence of Bcl-6 and MUM-1 expression&#44; leading to a diagnosis of chronic lymphocytic leukemia&#46; The definitive diagnosis was thus systemic DLBCL with secondary cutaneous involvement due to a previous unknown transformation of chronic lymphocytic leukemia &#40;Richter syndrome or transformation&#41;&#46; The patient was treated with R-CHOP&#44; which initially resulted in a reduction in the size of the skin tumor and the lymph nodes&#46; However&#44; he died 4 months later due to neutropenic sepsis&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">DLBCL is a heterogeneous entity with multiple clinical and pathologic variants&#46; It is most common in individuals aged over 60 years&#46; It is also the most common type of lymphoma in patients with human immunodeficiency virus infection&#46; Because of its high proliferative activity&#44; it is sometimes diagnosed earlier than other more indolent lymphomas&#46; Immunophenotypically&#44; all DLBCLs express B-cell markers&#44; and other markers are expressed in a variable proportion of tumors &#40;Bcl-6 in 60&#37;-90&#37;&#44; MUM-1 in 35&#37;-65&#37;&#44; and Bcl-2 in 50&#37;&#41;&#46; Although Bcl-2 is expressed in over 60&#37; of tumors&#44; <span class="elsevierStyleItalic">Bcl-2</span> rearrangement is observed in under 20&#37; of cases&#44; suggesting the existence of other genetic pathways for the expression of this protein&#46; There has also been a description of a double-hit variant involving both <span class="elsevierStyleItalic">Bcl-2</span> and <span class="elsevierStyleItalic">Myc</span> rearrangement associated with worse prognosis and a greater tendency for secondary cutaneous involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The primary cutaneous variant of DLBCL&#44; primary cutaneous diffuse large B-cell lymphoma &#40;PCDLBCL&#41;&#44; leg type&#44; is one of the 3 most common types of primary cutaneous B-cell lymphomas&#44; together with primary cutaneous follicle center lymphoma and marginal zone B-cell lymphoma&#46; PCDLBCL&#44; leg-type presents as solitary or multiple violaceous plaques that typically occur on the legs of elderly patients&#46; It is by far the primary cutaneous B-cell lymphoma with the worst prognosis&#44; with a 5-year survival of 50&#37;&#46; It is therefore essential to initiate treatment with multi-agent chemotherapy and rituximab as early as possible&#46;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Because the immunohistochemical markers are the same for PCDLBCL&#44; leg type and its systemic variant&#44; DLBCL&#44; according to the current WHO-EORTC criteria&#44;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">5</span></a> definitive diagnosis should be based on the presence or absence of extracutaneous disease at diagnosis&#46; Testicular B-cell lymphoma can also affect the skin and its pathologic and immunophenotypic characteristics are indistinguishable from those of PCDLBCL&#44; leg type&#59; routine testicular examination is therefore recommended in appropriate cases&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">We have reported a case of systemic DLBCL diagnosed following the detection of a cutaneous lesion without other signs or symptoms&#46; One particularly interesting aspect of this case was the presence of a solitary lesion that clinically resembled a primary cutaneous lymphoma&#46;</p></span>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Lloret-Ruiz C&#44; Mol&#233;s-Poveda P&#44; Barrado-Sol&#237;s N&#44; Gimeno-Carpio E&#46; Linfoma B difuso de c&#233;lulas grandes sist&#233;mico con afectaci&#243;n cut&#225;nea secundaria&#46; Actas Dermosifiliogr&#46; 2015&#59;106&#58;685-687&#46;</p>"
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Article information
ISSN: 15782190
Original language: English
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2016 October 26 21 47
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2016 August 0 1 1
2016 July 0 2 2
2016 June 3 6 9
2016 May 2 5 7
2016 April 6 3 9
2016 February 0 1 1
2016 January 0 3 3
2015 November 0 1 1
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Idiomas
Actas Dermo-Sifiliográficas
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?