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
, P. Molés-Poveda, N. Barrado-Solís, E. Gimeno-Carpio
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Servicio de Dermatología, Hospital Arnau de Vilanova, Valencia, Spain
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Lloret-Ruiz, P. Molés-Poveda, N. Barrado-Solís, E. Gimeno-Carpio" "autores" => array:4 [ 0 => array:4 [ "nombre" => "C." "apellidos" => "Lloret-Ruiz" "email" => array:1 [ 0 => "clloretruiz@gmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "P." "apellidos" => "Molés-Poveda" ] 2 => array:2 [ "nombre" => "N." "apellidos" => "Barrado-Solís" ] 3 => array:2 [ "nombre" => "E." "apellidos" => "Gimeno-Carpio" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Servicio de Dermatología, Hospital Arnau de Vilanova, Valencia, Spain" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Linfoma B difuso de células grandes sistémico con afectación cutánea secundaria" ] ] "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" => 1263 "Ancho" => 1682 "Tamanyo" => 314385 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Indurated erythematous-violaceous plaque.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Diffuse large B-cell lymphoma (DLBCL) is the most common type of systemic lymphoma and accounts for 40% of all new cases of this disease. Prognosis has improved considerably since the introduction of rituximab, which combined with chemotherapy is associated with a cure rate of up to 50%<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">1</span></a>; R-CHOP is the regimen of choice. Secondary cutaneous involvement is uncommon in DLBCL, although it has been observed in up to 20% of cases in some series.<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, erythematous-violaceous plaque-like lesion with a diameter of 7<span class="elsevierStyleHsp" style=""></span>×<span class="elsevierStyleHsp" style=""></span>4<span class="elsevierStyleHsp" style=""></span>cm on the right leg (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). The plaque had appeared 3 weeks earlier.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">A biopsy was performed to investigate the suspected diagnosis of primary cutaneous lymphoma, with results showing a diffuse, dense infiltration of large, highly pleomorphic atypical basophilic cells in the dermis (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Immunohistochemical stains were positive for B-cell markers (CD20, CD79a), negative for T-cell markers (CD3, CD5), and strongly positive for Bcl-2. Less intense staining was observed for Bcl-6 and MUM-1, and the results were negative for CD10 and CD30 (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A and B). Ki-67 staining showed high proliferative activity (90%). Fluorescent in situ hybridization was negative for <span class="elsevierStyleItalic">Bcl-2</span> rearrangement with the t(14;18) translocation.</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. Bone marrow biopsy showed an infiltration of small cells with positive B-cell markers. Unlike the skin biopsy, the bone marrow biopsy showed CD5 expression and an absence of Bcl-6 and MUM-1 expression, leading to a diagnosis of chronic lymphocytic leukemia. The definitive diagnosis was thus systemic DLBCL with secondary cutaneous involvement due to a previous unknown transformation of chronic lymphocytic leukemia (Richter syndrome or transformation). The patient was treated with R-CHOP, which initially resulted in a reduction in the size of the skin tumor and the lymph nodes. However, he died 4 months later due to neutropenic sepsis.</p><p id="par0025" class="elsevierStylePara elsevierViewall">DLBCL is a heterogeneous entity with multiple clinical and pathologic variants. It is most common in individuals aged over 60 years. It is also the most common type of lymphoma in patients with human immunodeficiency virus infection. Because of its high proliferative activity, it is sometimes diagnosed earlier than other more indolent lymphomas. Immunophenotypically, all DLBCLs express B-cell markers, and other markers are expressed in a variable proportion of tumors (Bcl-6 in 60%-90%, MUM-1 in 35%-65%, and Bcl-2 in 50%). Although Bcl-2 is expressed in over 60% of tumors, <span class="elsevierStyleItalic">Bcl-2</span> rearrangement is observed in under 20% of cases, suggesting the existence of other genetic pathways for the expression of this protein. 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.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">The primary cutaneous variant of DLBCL, primary cutaneous diffuse large B-cell lymphoma (PCDLBCL), leg type, is one of the 3 most common types of primary cutaneous B-cell lymphomas, together with primary cutaneous follicle center lymphoma and marginal zone B-cell lymphoma. PCDLBCL, leg-type presents as solitary or multiple violaceous plaques that typically occur on the legs of elderly patients. It is by far the primary cutaneous B-cell lymphoma with the worst prognosis, with a 5-year survival of 50%. It is therefore essential to initiate treatment with multi-agent chemotherapy and rituximab as early as possible.<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">4</span></a> Because the immunohistochemical markers are the same for PCDLBCL, leg type and its systemic variant, DLBCL, according to the current WHO-EORTC criteria,<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. Testicular B-cell lymphoma can also affect the skin and its pathologic and immunophenotypic characteristics are indistinguishable from those of PCDLBCL, leg type; routine testicular examination is therefore recommended in appropriate cases.<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. One particularly interesting aspect of this case was the presence of a solitary lesion that clinically resembled a primary cutaneous lymphoma.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Lloret-Ruiz C, Molés-Poveda P, Barrado-Solís N, Gimeno-Carpio E. Linfoma B difuso de células grandes sistémico con afectación cutánea secundaria. Actas Dermosifiliogr. 2015;106:685-687.</p>" ] ] "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1263 "Ancho" => 1682 "Tamanyo" => 314385 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Indurated erythematous-violaceous plaque.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1263 "Ancho" => 1685 "Tamanyo" => 473772 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Dense dermal infiltration of pleomorphic, atypical lymphocytes.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 970 "Ancho" => 2589 "Tamanyo" => 468966 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Positive immunohistochemical staining for CD20 (A) and Bcl-2 (B) (original magnification ×20).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0035" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Aggressive Lymphomas" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "G. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 8 | 8 | 16 |
| 2024 Octubre | 93 | 44 | 137 |
| 2024 Septiembre | 76 | 54 | 130 |
| 2024 Agosto | 111 | 63 | 174 |
| 2024 Julio | 102 | 36 | 138 |
| 2024 Junio | 121 | 41 | 162 |
| 2024 Mayo | 120 | 33 | 153 |
| 2024 Abril | 96 | 19 | 115 |
| 2024 Marzo | 89 | 36 | 125 |
| 2024 Febrero | 93 | 31 | 124 |
| 2024 Enero | 75 | 34 | 109 |
| 2023 Diciembre | 79 | 23 | 102 |
| 2023 Noviembre | 101 | 29 | 130 |
| 2023 Octubre | 72 | 19 | 91 |
| 2023 Septiembre | 89 | 34 | 123 |
| 2023 Agosto | 62 | 16 | 78 |
| 2023 Julio | 85 | 39 | 124 |
| 2023 Junio | 84 | 24 | 108 |
| 2023 Mayo | 123 | 24 | 147 |
| 2023 Abril | 85 | 25 | 110 |
| 2023 Marzo | 90 | 22 | 112 |
| 2023 Febrero | 86 | 24 | 110 |
| 2023 Enero | 67 | 26 | 93 |
| 2022 Diciembre | 88 | 42 | 130 |
| 2022 Noviembre | 51 | 25 | 76 |
| 2022 Octubre | 30 | 36 | 66 |
| 2022 Septiembre | 35 | 46 | 81 |
| 2022 Agosto | 36 | 30 | 66 |
| 2022 Julio | 22 | 33 | 55 |
| 2022 Junio | 22 | 22 | 44 |
| 2022 Mayo | 59 | 42 | 101 |
| 2022 Abril | 82 | 42 | 124 |
| 2022 Marzo | 76 | 52 | 128 |
| 2022 Febrero | 77 | 34 | 111 |
| 2022 Enero | 85 | 54 | 139 |
| 2021 Diciembre | 47 | 41 | 88 |
| 2021 Noviembre | 52 | 52 | 104 |
| 2021 Octubre | 49 | 47 | 96 |
| 2021 Septiembre | 33 | 41 | 74 |
| 2021 Agosto | 77 | 39 | 116 |
| 2021 Julio | 69 | 41 | 110 |
| 2021 Junio | 61 | 43 | 104 |
| 2021 Mayo | 46 | 29 | 75 |
| 2021 Abril | 82 | 45 | 127 |
| 2021 Marzo | 77 | 28 | 105 |
| 2021 Febrero | 62 | 36 | 98 |
| 2021 Enero | 49 | 28 | 77 |
| 2020 Diciembre | 36 | 27 | 63 |
| 2020 Noviembre | 22 | 21 | 43 |
| 2020 Octubre | 22 | 15 | 37 |
| 2020 Septiembre | 33 | 19 | 52 |
| 2020 Agosto | 24 | 22 | 46 |
| 2020 Julio | 21 | 12 | 33 |
| 2020 Junio | 33 | 37 | 70 |
| 2020 Mayo | 21 | 10 | 31 |
| 2020 Abril | 37 | 17 | 54 |
| 2020 Marzo | 29 | 17 | 46 |
| 2020 Febrero | 10 | 1 | 11 |
| 2020 Enero | 0 | 2 | 2 |
| 2019 Diciembre | 8 | 2 | 10 |
| 2019 Noviembre | 4 | 1 | 5 |
| 2019 Octubre | 1 | 0 | 1 |
| 2019 Septiembre | 8 | 3 | 11 |
| 2019 Agosto | 4 | 1 | 5 |
| 2019 Julio | 4 | 4 | 8 |
| 2019 Junio | 4 | 15 | 19 |
| 2019 Mayo | 5 | 44 | 49 |
| 2019 Abril | 3 | 1 | 4 |
| 2019 Marzo | 2 | 3 | 5 |
| 2019 Febrero | 6 | 0 | 6 |
| 2018 Diciembre | 4 | 0 | 4 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 8 | 1 | 9 |
| 2018 Agosto | 0 | 11 | 11 |
| 2018 Julio | 0 | 5 | 5 |
| 2018 Junio | 0 | 3 | 3 |
| 2018 Mayo | 0 | 10 | 10 |
| 2018 Abril | 0 | 9 | 9 |
| 2018 Marzo | 0 | 2 | 2 |
| 2018 Febrero | 49 | 11 | 60 |
| 2018 Enero | 68 | 10 | 78 |
| 2017 Diciembre | 48 | 10 | 58 |
| 2017 Noviembre | 23 | 11 | 34 |
| 2017 Octubre | 35 | 7 | 42 |
| 2017 Septiembre | 23 | 15 | 38 |
| 2017 Agosto | 33 | 4 | 37 |
| 2017 Julio | 26 | 12 | 38 |
| 2017 Junio | 34 | 21 | 55 |
| 2017 Mayo | 39 | 21 | 60 |
| 2017 Abril | 15 | 14 | 29 |
| 2017 Marzo | 8 | 7 | 15 |
| 2017 Febrero | 19 | 6 | 25 |
| 2017 Enero | 15 | 14 | 29 |
| 2016 Diciembre | 28 | 16 | 44 |
| 2016 Noviembre | 36 | 27 | 63 |
| 2016 Octubre | 26 | 21 | 47 |
| 2016 Septiembre | 0 | 7 | 7 |
| 2016 Agosto | 0 | 1 | 1 |
| 2016 Julio | 0 | 2 | 2 |
| 2016 Junio | 3 | 6 | 9 |
| 2016 Mayo | 2 | 5 | 7 |
| 2016 Abril | 6 | 3 | 9 |
| 2016 Febrero | 0 | 1 | 1 |
| 2016 Enero | 0 | 3 | 3 |
| 2015 Noviembre | 0 | 1 | 1 |
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