Case and Research Letters
Systemic Follicular Lymphoma With Cutaneous Manifestations and Exclusively Cutaneous Recurrence
Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea
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Palacios Abufón, E. Acebo Mariñas, J. Gardeazabal García, J.C. García-Ruiz" "autores" => array:4 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "Palacios Abufón" "email" => array:1 [ 0 => "andres.palacios.ib@gmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "E." "apellidos" => "Acebo Mariñas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "J." "apellidos" => "Gardeazabal García" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "J.C." "apellidos" => "García-Ruiz" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario de Cruces, Baracaldo, Vizcaya, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Hematología, Hospital Universitario de Cruces, Baracaldo, Vizcaya, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1338 "Ancho" => 993 "Tamanyo" => 389853 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bcl2<span class="elsevierStyleSup">+</span> lymphoid infiltrate.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Follicular lymphoma (FL) constitutes approximately 30% of all non-Hodgkin lymphomas in Western countries. Clinical presentation is typically in the form of lymphadenopathy, hepatomegaly, splenomegaly and bone marrow infiltration.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Extranodal involvement is less frequent than in large B-cell lymphoma<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> and does not appear to affect prognosis; this is not the case with B-cell lymphoma.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a patient diagnosed with FL who developed skin nodules in the course of her disease. The lymphoma recurred after treatment, but it was exclusively limited to the skin, an unusual observation in this disease.</p><p id="par0015" class="elsevierStylePara elsevierViewall">The patient was a 54-year-old woman who was referred to the hematology department in August 2004 for thrombocytopenia detected during a routine blood analysis (platelet count 70×10<span class="elsevierStyleSup">9</span>/L). Monoclonal B-cell lymphocytosis in the peripheral blood and bone marrow, enlarged subcentimeter abdominal lymph nodes and splenomegaly were observed during the study. The diagnosis was chronic B-cell lymphoproliferative syndrome and the patient was offered splenectomy, which she refused at that time. At follow-up in January 2007, enlarged mediastinal and retroperitoneal lymph nodes (≤5<span class="elsevierStyleHsp" style=""></span>cm in diameter) were observed, prompting the performance of diagnostic and therapeutic splenectomy. Histologic study of the spleen showed a proliferation of small lymphoid cells, with a micronodular growth pattern, predominantly in the germinal centers of the white pulp, with infiltration of the red pulp. The cells had a CD20<span class="elsevierStyleSup">+</span>, Bcl2<span class="elsevierStyleSup">+</span>, Bcl6<span class="elsevierStyleSup">+</span>, IgD<span class="elsevierStyleSup">−</span> CD23<span class="elsevierStyleSup">−</span>, cyclin D1<span class="elsevierStyleSup">−</span> and p53<span class="elsevierStyleSup">−</span> phenotype and a low proliferative index, findings which were consistent with the diagnosis of FL.</p><p id="par0020" class="elsevierStylePara elsevierViewall">In March 2007, the patient was referred to the dermatology department due to the gradual appearance of asymptomatic subcutaneous nodular lesions on the face, chest and back. The nodules had an erythematous-violacious appearance, were slightly raised, and had diameters ranging between 8<span class="elsevierStyleHsp" style=""></span>mm and 20<span class="elsevierStyleHsp" style=""></span>mm (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Biopsy of the lesions showed a lymphoid infiltrate composed of small monomorphous cells, compatible with FL infiltration (CD20<span class="elsevierStyleSup">+</span>, Bcl2<span class="elsevierStyleSup">+</span>, Bcl6<span class="elsevierStyleSup">+</span>) (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Reassessment of the disease at this time also demonstrated the development of lymphadenopathy, for which chemotherapy was started with a rituximab-CHOP schedule (cyclophosphamide, vincristine, adriamycin and prednisone). Complete remission was achieved after 6 cycles, with clearance of the subcutaneous nodules and the enlarged lymph nodes; subsequently, the patient was placed on maintenance therapy with rituximab administered every 3 months. In December 2009, the patient again presented with cutaneous lesions, predominantly facial, similar to those previously described; biopsy reconfirmed the diagnosis of infiltration by FL. The rituximab maintenance treatment ended in June 2010; the skin lesions were still stable and there was no evidence of disease spread to other areas (peripheral blood immunophenotyping and cervical-thoracic-abdomen CT scan were normal).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0025" class="elsevierStylePara elsevierViewall">FL is characterized by an indolent course and has no standardized treatment; a variety of approaches are used, including simple observation, radiotherapy, immunotherapy, polychemotherapy, maintenance treatment with rituximab,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> and even the application of various hematopoietic stem cell transplantation treatment modalities.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">As in other lymphoid malignancies, patients with FL can have extranodal involvement affecting the skin in up to 3.8% of cases.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Extranodal involvement in FL does not generally affect overall survival, unlike the case with large diffuse B-cell lymphoma; these 2 diseases constitute the majority of B-cell lymphomas in Spain.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> It is therefore important to rule out transformation of FL to more aggressive forms as these are associated with significantly decreased survival.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> For this reason, lesions should be biopsied whenever a patient experiences relapse or disease progression.</p><p id="par0035" class="elsevierStylePara elsevierViewall">Differential diagnosis is particularly important in primary cutaneous follicular center lymphoma, since it shares histologic features with FL. In addition to systemic involvement, Bcl2 negativity, which is common in cases primarily involving the skin, may be of use in differentiating the 2 diseases.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">FL usually responds well to treatment, although relapses are common and usually affect the lymph nodes, bone marrow or peripheral blood.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> What is unusual is recurrence being limited to the skin, as in our patient. At present, and after more than 6 years of follow-up, the patient's disease remains limited to the skin. The persistence of cutaneous disease reflects a lack of complete control of the disease, and it is therefore likely that the patient will develop systemic disease in the future.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Palacios Abufón A, et al. Linfoma folicular sistémico con afectación cutánea y recidiva únicamente cutánea. Actas Dermosifiliogr. 2012;103:253–55.</p>" ] ] "multimedia" => array:2 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 830 "Ancho" => 993 "Tamanyo" => 112437 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Nodule on the chest.</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" => 1338 "Ancho" => 993 "Tamanyo" => 389853 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Bcl2<span class="elsevierStyleSup">+</span> lymphoid infiltrate.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Follicular lymphomas" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "U. 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Blokx" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1200/JCO.2006.09.6396" "Revista" => array:6 [ "tituloSerie" => "J Clin Oncol" "fecha" => "2007" "volumen" => "25" "paginaInicial" => "1581" "paginaFinal" => "1587" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/17353548" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15782190/0000010300000003/v1_201304241257/S1578219012001059/v1_201304241257/en/main.assets" "Apartado" => array:4 [ "identificador" => "6169" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Cases and Research Letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15782190/0000010300000003/v1_201304241257/S1578219012001059/v1_201304241257/en/main.pdf?idApp=UINPBA000044&text.app=https://actasdermo.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219012001059?idApp=UINPBA000044" ]
| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 21 | 12 | 33 |
| 2024 Octubre | 177 | 65 | 242 |
| 2024 Septiembre | 152 | 25 | 177 |
| 2024 Agosto | 147 | 58 | 205 |
| 2024 Julio | 131 | 48 | 179 |
| 2024 Junio | 115 | 96 | 211 |
| 2024 Mayo | 128 | 46 | 174 |
| 2024 Abril | 114 | 29 | 143 |
| 2024 Marzo | 179 | 29 | 208 |
| 2024 Febrero | 181 | 38 | 219 |
| 2024 Enero | 462 | 38 | 500 |
| 2023 Diciembre | 394 | 17 | 411 |
| 2023 Noviembre | 545 | 24 | 569 |
| 2023 Octubre | 679 | 39 | 718 |
| 2023 Septiembre | 617 | 42 | 659 |
| 2023 Agosto | 673 | 17 | 690 |
| 2023 Julio | 645 | 35 | 680 |
| 2023 Junio | 502 | 27 | 529 |
| 2023 Mayo | 312 | 32 | 344 |
| 2023 Abril | 154 | 19 | 173 |
| 2023 Marzo | 134 | 39 | 173 |
| 2023 Febrero | 278 | 22 | 300 |
| 2023 Enero | 501 | 34 | 535 |
| 2022 Diciembre | 260 | 49 | 309 |
| 2022 Noviembre | 214 | 28 | 242 |
| 2022 Octubre | 346 | 30 | 376 |
| 2022 Septiembre | 402 | 61 | 463 |
| 2022 Agosto | 238 | 53 | 291 |
| 2022 Julio | 128 | 37 | 165 |
| 2022 Junio | 58 | 35 | 93 |
| 2022 Mayo | 154 | 42 | 196 |
| 2022 Abril | 254 | 37 | 291 |
| 2022 Marzo | 321 | 61 | 382 |
| 2022 Febrero | 234 | 36 | 270 |
| 2022 Enero | 221 | 45 | 266 |
| 2021 Diciembre | 122 | 39 | 161 |
| 2021 Noviembre | 183 | 48 | 231 |
| 2021 Octubre | 142 | 50 | 192 |
| 2021 Septiembre | 111 | 35 | 146 |
| 2021 Agosto | 104 | 45 | 149 |
| 2021 Julio | 107 | 29 | 136 |
| 2021 Junio | 106 | 28 | 134 |
| 2021 Mayo | 76 | 38 | 114 |
| 2021 Abril | 181 | 70 | 251 |
| 2021 Marzo | 103 | 27 | 130 |
| 2021 Febrero | 96 | 26 | 122 |
| 2021 Enero | 53 | 19 | 72 |
| 2020 Diciembre | 62 | 16 | 78 |
| 2020 Noviembre | 48 | 16 | 64 |
| 2020 Octubre | 44 | 12 | 56 |
| 2020 Septiembre | 32 | 10 | 42 |
| 2020 Agosto | 48 | 23 | 71 |
| 2020 Julio | 23 | 15 | 38 |
| 2020 Junio | 37 | 32 | 69 |
| 2020 Mayo | 23 | 17 | 40 |
| 2020 Abril | 27 | 21 | 48 |
| 2020 Marzo | 25 | 15 | 40 |
| 2020 Febrero | 6 | 1 | 7 |
| 2020 Enero | 4 | 0 | 4 |
| 2019 Diciembre | 8 | 4 | 12 |
| 2019 Noviembre | 4 | 0 | 4 |
| 2019 Octubre | 0 | 20 | 20 |
| 2019 Septiembre | 8 | 0 | 8 |
| 2019 Agosto | 4 | 1 | 5 |
| 2019 Julio | 2 | 3 | 5 |
| 2019 Junio | 6 | 2 | 8 |
| 2019 Mayo | 3 | 18 | 21 |
| 2019 Marzo | 2 | 7 | 9 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 3 | 0 | 3 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 2 | 0 | 2 |
| 2018 Septiembre | 3 | 0 | 3 |
| 2018 Marzo | 5 | 0 | 5 |
| 2018 Febrero | 82 | 3 | 85 |
| 2018 Enero | 84 | 4 | 88 |
| 2017 Diciembre | 70 | 7 | 77 |
| 2017 Noviembre | 47 | 3 | 50 |
| 2017 Octubre | 63 | 5 | 68 |
| 2017 Septiembre | 68 | 2 | 70 |
| 2017 Agosto | 83 | 8 | 91 |
| 2017 Julio | 58 | 4 | 62 |
| 2017 Junio | 78 | 7 | 85 |
| 2017 Mayo | 67 | 5 | 72 |
| 2017 Abril | 66 | 5 | 71 |
| 2017 Marzo | 51 | 11 | 62 |
| 2017 Febrero | 44 | 11 | 55 |
| 2017 Enero | 43 | 8 | 51 |
| 2016 Diciembre | 49 | 7 | 56 |
| 2016 Noviembre | 110 | 9 | 119 |
| 2016 Octubre | 62 | 7 | 69 |
| 2016 Septiembre | 72 | 19 | 91 |
| 2016 Agosto | 60 | 11 | 71 |
| 2016 Julio | 36 | 9 | 45 |
| 2016 Junio | 9 | 15 | 24 |
| 2016 Mayo | 6 | 13 | 19 |
| 2016 Abril | 9 | 12 | 21 |
| 2016 Marzo | 10 | 2 | 12 |
| 2016 Febrero | 12 | 2 | 14 |
| 2016 Enero | 9 | 0 | 9 |
| 2015 Diciembre | 9 | 2 | 11 |
| 2015 Noviembre | 42 | 2 | 44 |
| 2015 Octubre | 40 | 0 | 40 |
| 2015 Septiembre | 12 | 6 | 18 |
| 2015 Agosto | 10 | 5 | 15 |
| 2015 Julio | 97 | 9 | 106 |
| 2015 Junio | 74 | 6 | 80 |
| 2015 Mayo | 100 | 10 | 110 |
| 2015 Abril | 81 | 9 | 90 |
| 2015 Marzo | 84 | 6 | 90 |
| 2015 Febrero | 114 | 17 | 131 |
| 2015 Enero | 87 | 20 | 107 |
| 2014 Diciembre | 86 | 7 | 93 |
| 2014 Noviembre | 58 | 13 | 71 |
| 2014 Octubre | 79 | 10 | 89 |
| 2014 Septiembre | 69 | 9 | 78 |
| 2014 Agosto | 73 | 12 | 85 |
| 2014 Julio | 77 | 23 | 100 |
| 2014 Junio | 103 | 14 | 117 |
| 2014 Mayo | 100 | 17 | 117 |
| 2014 Abril | 62 | 14 | 76 |
| 2014 Marzo | 33 | 7 | 40 |
| 2014 Febrero | 30 | 5 | 35 |
| 2014 Enero | 25 | 10 | 35 |
| 2013 Diciembre | 13 | 10 | 23 |
| 2013 Noviembre | 17 | 10 | 27 |
| 2013 Octubre | 16 | 6 | 22 |
| 2013 Septiembre | 9 | 5 | 14 |
| 2013 Agosto | 10 | 11 | 21 |
| 2013 Julio | 11 | 19 | 30 |
| 2013 Junio | 13 | 20 | 33 |
| 2013 Mayo | 17 | 11 | 28 |
| 2013 Abril | 15 | 8 | 23 |
| 2013 Marzo | 21 | 12 | 33 |
| 2013 Febrero | 40 | 7 | 47 |
| 2013 Enero | 88 | 8 | 96 |
| 2012 Diciembre | 32 | 5 | 37 |
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