Case and Research Letters
Cerebral Involvement as the First Extracutaneous Manifestation of Mycosis Fungoides
Micosis fungoide con afectación cerebral como primera manifestación extracutánea
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B: Biopsia de tejido cerebral que muestra un infiltrado linfoide de predominio perivascular (HE, ×200). C: Tinción inmunohistoquímica que pone de manifiesto el infiltrado linfocitario CD4+ en tejido cerebral (CD4, ×100). D: Expresión del antígeno CD30 en escasas células linfoides del tejido cerebral (CD30, ×40).</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "A. de Quintana-Sancho, E. Acebo-Mariñas, J. Gardeazabal-García, A. Aperribay-Esparza" "autores" => array:4 [ 0 => array:2 [ "nombre" => "A." "apellidos" => "de Quintana-Sancho" ] 1 => array:2 [ "nombre" => "E." "apellidos" => "Acebo-Mariñas" ] 2 => array:2 [ "nombre" => "J." "apellidos" => "Gardeazabal-García" ] 3 => array:2 [ "nombre" => "A." "apellidos" => "Aperribay-Esparza" ] ] ] ] ] "idiomaDefecto" => "es" "Traduccion" => array:1 [ "en" => array:9 [ "pii" => "S157821901500205X" "doi" => "10.1016/j.adengl.2015.07.007" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "en" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S157821901500205X?idApp=UINPBA000044" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731015002148?idApp=UINPBA000044" "url" => "/00017310/0000010600000008/v1_201510020746/S0001731015002148/v1_201510020746/es/main.assets" ] ] "itemAnterior" => array:19 [ "pii" => "S1578219015002140" "issn" => "15782190" "doi" => "10.1016/j.adengl.2015.07.016" "estado" => "S300" "fechaPublicacion" => "2015-10-01" "aid" => "1183" "copyright" => "Elsevier España, S.L.U. and AEDV" "documento" => "simple-article" "crossmark" => 1 "subdocumento" => "cor" "cita" => "Actas Dermosifiliogr. 2015;106:692-3" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:2 [ "total" => 1363 "formatos" => array:3 [ "EPUB" => 53 "HTML" => 553 "PDF" => 757 ] ] "en" => array:10 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letters</span>" "titulo" => "Thrombocytopenia Probably Induced by Acitretin" "tienePdf" => "en" "tieneTextoCompleto" => "en" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "692" "paginaFinal" => "693" ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Trombocitopenia en probable relación con acitretina" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M. García-Arpa, M. López-Nieto, J.L. Santiago Sánchez-Mateos, M.P. Sánchez-Caminero" "autores" => array:4 [ 0 => array:2 [ "nombre" => "M." "apellidos" => "García-Arpa" ] 1 => array:2 [ "nombre" => "M." "apellidos" => "López-Nieto" ] 2 => array:2 [ "nombre" => "J.L." "apellidos" => "Santiago Sánchez-Mateos" ] 3 => array:2 [ "nombre" => "M.P." "apellidos" => "Sánchez-Caminero" ] ] ] ] ] "idiomaDefecto" => "en" "Traduccion" => array:1 [ "es" => array:9 [ "pii" => "S0001731015002136" "doi" => "10.1016/j.ad.2015.03.010" "estado" => "S300" "subdocumento" => "" "abierto" => array:3 [ "ES" => true "ES2" => true "LATM" => true ] "gratuito" => true "lecturas" => array:1 [ "total" => 0 ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731015002136?idApp=UINPBA000044" ] ] "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1578219015002140?idApp=UINPBA000044" "url" => "/15782190/0000010600000008/v1_201510011018/S1578219015002140/v1_201510011018/en/main.assets" ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letters</span>" "titulo" => "Cerebral Involvement as the First Extracutaneous Manifestation of Mycosis Fungoides" "tieneTextoCompleto" => true "saludo" => "To the Editor:" "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "693" "paginaFinal" => "695" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "A. de Quintana-Sancho, E. Acebo-Mariñas, J. Gardeazabal-García, A. Aperribay-Esparza" "autores" => array:4 [ 0 => array:4 [ "nombre" => "A." "apellidos" => "de Quintana-Sancho" "email" => array:1 [ 0 => "adriandeq@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" => "A." "apellidos" => "Aperribay-Esparza" "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 Cruces, Barakaldo, Vizcaya, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Micosis fungoide con afectación cerebral como primera manifestación extracutánea" ] ] "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" => 1833 "Ancho" => 2519 "Tamanyo" => 1292637 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Diffuse white matter signal abnormality, especially in the frontal and periventricular regions (arrows). B, Brain tissue biopsy showing a predominantly perivascular lymphoid infiltrate (hematoxylin-eosin, ×200). C, Immunohistochemical staining showing the CD4<span class="elsevierStyleSup">+</span> lymphocytic infiltrate in brain tissue (CD4, ×100). D, Expression of CD30 antigen in sparse lymphoid cells of brain tissue (CD30, ×40).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Mycosis fungoides is the most common cutaneous lymphoma. The probability of systemic involvement depends on disease extension and is very low during the early stages, when the clinical course is usually indolent. However, the risk of extracutaneous disease 20 years after diagnosis is 10% in patients with generalized plaques and 35.5% in patients with tumorous lesions.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> The most common finding is enlarged regional lymph nodes, although any organ can be affected, especially the lungs, spleen, liver, and gastrointestinal tract.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">2</span></a> Central nervous system (CNS) involvement is very uncommon, even more so in the absence of extracutaneous disease.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The patient was a 47-year-old woman with erythematous-desquamative lesions that first appeared 6 years previously and were diagnosed as stage 1b mycosis fungoides after 4 inconclusive biopsies. During a 4-year follow-up period she received treatment with potent topical corticosteroids, psoralen-UV-A, interferon, and oral bexarotene. During the last year, the patient developed tumorous lesions (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), with 14% Sézary cells (<<span class="elsevierStyleHsp" style=""></span>1000/μL) in peripheral blood and unremarkable findings on a computed tomography (CT) scan of the chest, abdomen, and pelvis (stage IIb). The dose of oral bexarotene was increased and local radiation therapy started, and a partial response was observed. Skin biopsy of a rapidly growing tumorous lesion revealed large cell transformation with marked positivity for CD30 (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B-D) that had not been present in the previous biopsies. A few months later the patient complained of recent memory loss and depression. The physical examination revealed nystagmus and dysmetria. An emergency CT scan of the brain ruled out acute disease. Similarly, a subsequent CT scan of the chest, abdomen, and pelvis revealed no significant abnormalities; nevertheless, the patient was admitted to hospital. Magnetic resonance imaging of the brain revealed diffuse white matter signal abnormalities, especially in the frontal region (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A), which was compatible with infectious encephalitis. Analysis of cerebrospinal fluid revealed pleocytosis dominated by T lymphocytes with no atypia. Culture was negative. Biopsy of brain tissue revealed infiltration by CD4<span class="elsevierStyleSup">+</span>CD8<span class="elsevierStyleSup">–</span> T-cell lymphoma (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B and C) with no large cell transformation and isolated CD30<span class="elsevierStyleSup">+</span> cells (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>D). Chemotherapy was started according to the BAM protocol (carmustine, methotrexate, and cytarabine). However, the disease progressed, with biopsy-confirmed kidney metastases and multiple pulmonary nodules compatible with metastasis. Large cell transformation was not observed in brain tissue or kidney tissue; therefore, the case was considered to be a localized phenomenon affecting the skin. Unfortunately, the patient died from respiratory insufficiency 3 months after the onset of neurological symptoms.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">In contrast with other cutaneous lymphomas, such as aggressive epidermotropic CD8<span class="elsevierStyleSup">+</span> T-cell lymphoma, involvement of the CNS as a result of mycosis fungoides is exceptional, accounting for 1.6% of a series of 187 patients.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> However, CNS involvement was found in 11% to 14% of autopsies performed on patients who died of mycosis fungoides.<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> Some authors report meningeal involvement to be the most common finding,<a class="elsevierStyleCrossRef" href="#bib0070"><span class="elsevierStyleSup">4</span></a> whereas others claim that intraparenchymal brain involvement is the most common, with both forms able to occur simultaneously.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> The male-to-female ratio for this disease is 4:1, and in most cases, symptoms appear between 3 and 5 years after diagnosis of mycosis fungoides.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a> The most frequent symptoms include confusion and depression, which are associated with lethargy and nausea. Optic and facial nerve involvement is common, as are gait disorders. The prognosis is poor, with a mean survival of 4.5 months after diagnosis.<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">5</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">As for risk factors associated with CNS involvement, the series of Stein et al.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> showed that patients with at least 2 of 4 risk factors (T3-T4, N3, M1, and B1) had a 1 in 6 risk of CNS involvement at 10 years of follow-up compared with a 1 in a 100 risk in patients with 1 or none of the 4 risk factors.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The literature contains barely 10 cases of mycosis fungoides affecting the CNS associated with large cell transformation.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,7,8</span></a> Patients with mycosis fungoides and large cell transformation have a 5% probability of CNS involvement, compared with 1.6% of patients with no large cell transformation.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> Furthermore, while patients with large cell transformation usually present extracutaneous symptoms before cerebral involvement (lymph nodes and viscera), some patients with large cell transformation develop cerebral metastasis without previously having developed extracutaneous symptoms, as in the case we present.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> These patients should have an annual cerebral CT scan.<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">8</span></a> As for CD30 expression, some studies show a better prognosis in patients with large cell transformation and CD30<span class="elsevierStyleSup">+</span> cells.<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Mycosis fungoides very rarely affects the CNS. Patients with large cell transformation are usually at increased risk, sometimes without previously having developed extracutaneous symptoms. Such patients should undergo radiological evaluation, and physicians should be on the alert for neurological symptoms.</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: de Quintana-Sancho A, Acebo-Mariñas E, Gardeazabal-García J, Aperribay-Esparza A. Micosis fungoide con afectación cerebral como primera manifestación extracutánea. Actas Dermosifiliogr. 2015;106:693–695.</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" => 1833 "Ancho" => 2519 "Tamanyo" => 1512614 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Mycosis fungoides in the tumor phase. B, Biopsy (detail) of a tumorous lesion. Note the diffuse large cell lymphoid infiltrate in the dermis (hematoxylin-eosin, original magnification, ×400). C, Immunohistochemical staining showing predominance of CD4<span class="elsevierStyleSup">+</span> T lymphocytes (CD4, ×400). D, Expression of CD30 antigen in cells of the lymphoid infiltrate (CD30, ×400).</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" => 1833 "Ancho" => 2519 "Tamanyo" => 1292637 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Diffuse white matter signal abnormality, especially in the frontal and periventricular regions (arrows). B, Brain tissue biopsy showing a predominantly perivascular lymphoid infiltrate (hematoxylin-eosin, ×200). C, Immunohistochemical staining showing the CD4<span class="elsevierStyleSup">+</span> lymphocytic infiltrate in brain tissue (CD4, ×100). D, Expression of CD30 antigen in sparse lymphoid cells of brain tissue (CD30, ×40).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:10 [ 0 => array:3 [ "identificador" => "bib0055" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinical characteristics and outcome of patients with extracutaneous mycosis fungoides" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "E.C. De Coninck" 1 => "Y.H. Kim" 2 => "A. Varghese" 3 => "R.T. Hoppe" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1200/jco.2001.19.3.779" "Revista" => array:6 [ "tituloSerie" => "J Clin Oncol." 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 15 | 6 | 21 |
| 2024 Octubre | 109 | 46 | 155 |
| 2024 Septiembre | 120 | 38 | 158 |
| 2024 Agosto | 147 | 63 | 210 |
| 2024 Julio | 109 | 45 | 154 |
| 2024 Junio | 116 | 38 | 154 |
| 2024 Mayo | 101 | 37 | 138 |
| 2024 Abril | 102 | 30 | 132 |
| 2024 Marzo | 101 | 46 | 147 |
| 2024 Febrero | 69 | 39 | 108 |
| 2024 Enero | 75 | 29 | 104 |
| 2023 Diciembre | 75 | 28 | 103 |
| 2023 Noviembre | 75 | 31 | 106 |
| 2023 Octubre | 72 | 26 | 98 |
| 2023 Septiembre | 90 | 30 | 120 |
| 2023 Agosto | 66 | 21 | 87 |
| 2023 Julio | 87 | 32 | 119 |
| 2023 Junio | 68 | 31 | 99 |
| 2023 Mayo | 95 | 31 | 126 |
| 2023 Abril | 57 | 22 | 79 |
| 2023 Marzo | 63 | 17 | 80 |
| 2023 Febrero | 73 | 25 | 98 |
| 2023 Enero | 52 | 35 | 87 |
| 2022 Diciembre | 101 | 55 | 156 |
| 2022 Noviembre | 50 | 40 | 90 |
| 2022 Octubre | 56 | 34 | 90 |
| 2022 Septiembre | 38 | 41 | 79 |
| 2022 Agosto | 39 | 45 | 84 |
| 2022 Julio | 38 | 37 | 75 |
| 2022 Junio | 46 | 31 | 77 |
| 2022 Mayo | 54 | 37 | 91 |
| 2022 Abril | 104 | 61 | 165 |
| 2022 Marzo | 119 | 61 | 180 |
| 2022 Febrero | 90 | 41 | 131 |
| 2022 Enero | 75 | 43 | 118 |
| 2021 Diciembre | 52 | 61 | 113 |
| 2021 Noviembre | 72 | 61 | 133 |
| 2021 Octubre | 130 | 58 | 188 |
| 2021 Septiembre | 58 | 41 | 99 |
| 2021 Agosto | 75 | 44 | 119 |
| 2021 Julio | 43 | 36 | 79 |
| 2021 Junio | 36 | 49 | 85 |
| 2021 Mayo | 59 | 38 | 97 |
| 2021 Abril | 103 | 63 | 166 |
| 2021 Marzo | 83 | 34 | 117 |
| 2021 Febrero | 60 | 35 | 95 |
| 2021 Enero | 49 | 26 | 75 |
| 2020 Diciembre | 45 | 25 | 70 |
| 2020 Noviembre | 43 | 31 | 74 |
| 2020 Octubre | 20 | 20 | 40 |
| 2020 Septiembre | 55 | 24 | 79 |
| 2020 Agosto | 37 | 26 | 63 |
| 2020 Julio | 41 | 12 | 53 |
| 2020 Junio | 43 | 45 | 88 |
| 2020 Mayo | 32 | 28 | 60 |
| 2020 Abril | 36 | 22 | 58 |
| 2020 Marzo | 37 | 18 | 55 |
| 2020 Febrero | 1 | 8 | 9 |
| 2020 Enero | 6 | 15 | 21 |
| 2019 Diciembre | 8 | 4 | 12 |
| 2019 Noviembre | 4 | 1 | 5 |
| 2019 Octubre | 0 | 5 | 5 |
| 2019 Septiembre | 4 | 1 | 5 |
| 2019 Agosto | 4 | 3 | 7 |
| 2019 Julio | 4 | 3 | 7 |
| 2019 Junio | 4 | 29 | 33 |
| 2019 Mayo | 7 | 34 | 41 |
| 2019 Abril | 4 | 14 | 18 |
| 2019 Marzo | 4 | 5 | 9 |
| 2019 Febrero | 0 | 3 | 3 |
| 2019 Enero | 4 | 3 | 7 |
| 2018 Diciembre | 4 | 0 | 4 |
| 2018 Noviembre | 2 | 0 | 2 |
| 2018 Octubre | 3 | 0 | 3 |
| 2018 Septiembre | 9 | 0 | 9 |
| 2018 Mayo | 0 | 1 | 1 |
| 2018 Febrero | 24 | 3 | 27 |
| 2018 Enero | 26 | 7 | 33 |
| 2017 Diciembre | 32 | 8 | 40 |
| 2017 Noviembre | 18 | 2 | 20 |
| 2017 Octubre | 27 | 5 | 32 |
| 2017 Septiembre | 18 | 11 | 29 |
| 2017 Agosto | 24 | 3 | 27 |
| 2017 Julio | 19 | 8 | 27 |
| 2017 Junio | 24 | 9 | 33 |
| 2017 Mayo | 29 | 15 | 44 |
| 2017 Abril | 15 | 5 | 20 |
| 2017 Marzo | 11 | 73 | 84 |
| 2017 Febrero | 19 | 11 | 30 |
| 2017 Enero | 10 | 11 | 21 |
| 2016 Diciembre | 19 | 13 | 32 |
| 2016 Noviembre | 25 | 18 | 43 |
| 2016 Octubre | 21 | 19 | 40 |
| 2016 Septiembre | 0 | 1 | 1 |
| 2016 Julio | 1 | 1 | 2 |
| 2016 Junio | 5 | 2 | 7 |
| 2016 Abril | 4 | 0 | 4 |
| 2016 Marzo | 1 | 0 | 1 |
| 2015 Diciembre | 0 | 2 | 2 |
| 2015 Noviembre | 0 | 1 | 1 |
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