Case and Research Letter
Nasal-Type Extranodal Natural Killer T-Cell Lymphoma
Linfoma de células NK/T extranodal nasal
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Estudio piloto" ] ] "contieneTextoCompleto" => array:1 [ "en" => true ] "contienePdf" => array:1 [ "en" => true ] "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" => 2252 "Ancho" => 3000 "Tamanyo" => 503585 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Ultrasound images comparing color Doppler in healthy skin and skin with capillary malformations and X flow in healthy skin and skin with capillary malformations. CM indicates capillary malformations.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "I. Salgüero Fernández, F. Alfageme Roldán, G. Roustan Gullón" "autores" => array:3 [ 0 => array:2 [ "nombre" => "I." "apellidos" => "Salgüero Fernández" ] 1 => array:2 [ "nombre" => "F." 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Moreno-Suárez, P. Bautista Martínez, F. Jiménez Burgos" "autores" => array:3 [ 0 => array:4 [ "nombre" => "F." "apellidos" => "Moreno-Suárez" "email" => array:1 [ 0 => "fatimacordoba@hotmail.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" => "P." "apellidos" => "Bautista Martínez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "F." "apellidos" => "Jiménez Burgos" "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 Santa Bárbara, Puertollano, Ciudad Real, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Santa Bárbara, Puertollano, Ciudad Real, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Linfoma de células NK/T extranodal nasal" ] ] "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" => 1234 "Ancho" => 1400 "Tamanyo" => 311872 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Well-defined, infiltrated reddish plaques affecting the right cheek and lower eyelid of the right eye, causing partial occlusion of the eye. B, Infiltrated erythematous plaques on the tip of the nose and border of the right nares (arrows).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Extranodal natural killer/T-cell lymphoma (NKTCL), nasal type, is a non-Hodgkin lymphoma that preferentially affects the nasal cavity and paranasal sinuses. The appearance of this disease as a primary neoplasm at other sites, such as the skin, upper respiratory tract, gastrointestinal tract, or testicles, has been reported. Primary NKTCLs of the skin are rare; secondary infiltration of the skin adjacent to a lymphoma arising in the nasal mucosa or in the paranasal sinuses is more common. This is a rare disease, but it has a rapid and aggressive clinical course. It is characterized by central facial lesions that are locally destructive and progress to necrosis. The lack of specificity of the initial clinical characteristics can delay diagnosis.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">We present the case of a 77-year-old man who reported a 2-month history of right-sided rhinorrhea and rapidly growing skin lesions on the right side of the face. On examination, infiltrated erythematous plaques with well-defined borders were observed on the lower eyelid of the right eye and right cheek (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A), and other smaller plaques on the tip of the nose and border of the right nares (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). Histology revealed a dense infiltrate of lymphocytic with atypical morphological features and an angiocentric distribution (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, A and B). On immunohistochemistry, there were T lymphocytes positive for CD3, CD56, and granzyme B (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>, A-C), and the presence of Epstein-Barr virus (EBV) (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>D) was confirmed on EBER (EBV encoded RNAs) stain. Complete blood count, biochemistry, and bone marrow biopsy were normal. Computed tomography revealed a hypervascular tumor affecting the right orbit, nose, and cheek. Based on these findings we made a diagnosis of stage IA extranodal NKTCL, nasal-type. Despite the early diagnosis and treatment with radiotherapy and chemotherapy, the patient died a year later.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The term extranodal NKTCL, nasal-type, was first used in the year 2001 by the World Health Organization, replacing the term angiocentric lymphoma.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> Other names by which this disease has been known in the past include lethal midline granuloma, necrotizing midfacial lesion, polymorphous reticulosis, angiocentric immunoproliferative lesion, Stewart granuloma, granuloma gangrenescens, gangrenous proliferative rhinitis, and sinonasal lymphoma. Extranodal NKTCL, nasal-type, accounts for 7% to 10% of non-Hodgkin lymphomas in Asia and South America, but only 1% in Europe and North America. It mainly affects individuals in their fifth decade of life and is more prevalent in men. As in our patient, the disease usually presents initially with rhinorrhea or a purulent nasal secretion, progressing subsequently to lesions with a tendency to ulceration and necrosis secondary to infiltration of the surrounding skin.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–5</span></a> The prognosis is very poor, even with early diagnosis, as in our patient, with a mean survival of less than 12 months.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,7</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">The differential diagnosis must include entities that affect the facial midline, of infectious (<span class="elsevierStyleItalic">Leishmania</span>, paracoccidioidomycosis, histoplasmosis, lepromatous leprosy, mycobacteriosis, mucormycosis), autoimmune (Wegener granulomatosis, lupus), or toxic (cocaine sniffing) etiology.<a class="elsevierStyleCrossRefs" href="#bib0075"><span class="elsevierStyleSup">5,8</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The etiology of this lymphoma is unknown, but the presence of EBV is an almost constant finding, with detection of viral RNA in the diseased lymphocytes by in situ hybridization using probes marked with Epstein-Barr-encoded small RNAs (EBER); this would suggest a major etiologic and pathogenic role for this virus in the development of this disease.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5,8</span></a> The majority of cells that make up this neoplasm are natural killer cells and peripheral T cells with a cytotoxic phenotype. Histologically, dense infiltrates of atypical lymphocytes are observed in the dermis and subcutaneous cellular tissue. These are small-to-medium sized cells with pale cytoplasm and atypical nuclei with clumped chromatin and irregular borders. There is marked angiocentricity and angiodestruction associated with extensive necrosis. Immunohistochemically, the neoplastic cells are positive for CD2, cytoplasmic CD3, CD56, and cytotoxic proteins (granzyme B, T-cell intracellular antigen [TIA]-1, and perforin).<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">Treatment of NKTCL is based on radiotherapy for localized disease, though this is usually combined with chemotherapy. If the patient is initially managed using chemotherapy, palliative or coadjuvant radiotherapy is recommended. The poor response to chemotherapy may be due to the expression of multidrug resistance genes. Allogenic bone marrow transplant has been proposed in selected patients. Because of the intrinsically aggressive nature of this type of lymphoma, treatment does not reduce mortality in the majority of cases.<a class="elsevierStyleCrossRefs" href="#bib0095"><span class="elsevierStyleSup">9,10</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">Although this disease is rare in our setting, extranodal NKTCL, nasal-type, must be included in the differential diagnosis of skin lesions that affect the midfacial region and present with nasal symptoms, in order to avoid delayed diagnosis and treatment.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0040" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Moreno-Suárez F, Bautista Martínez P, Jiménez Burgos F. Linfoma de células NK/T extranodal nasal. Actas Dermosifiliogr. 2017;108:687–689</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" => 1234 "Ancho" => 1400 "Tamanyo" => 311872 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Well-defined, infiltrated reddish plaques affecting the right cheek and lower eyelid of the right eye, causing partial occlusion of the eye. B, Infiltrated erythematous plaques on the tip of the nose and border of the right nares (arrows).</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" => 932 "Ancho" => 1765 "Tamanyo" => 447695 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A, Dense lymphocytic infiltrate with a perivascular distribution in the mid and deep dermis, extending partially towards the epidermis and hair follicles. Edematous superficial dermis. Hyperplastic epidermis with preserved architecture and maturation. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×100. B, Population of lymphocytes with scant, pale eosinophilic cytoplasm and atypical nuclei with clumped chromatin and irregular borders. Hematoxylin and eosin, original magnification<span class="elsevierStyleHsp" style=""></span>×400).</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" => 1421 "Ancho" => 1800 "Tamanyo" => 649500 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">A, A CD3<span class="elsevierStyleSup">+</span> lymphoid population is present in the superficial and mid dermis, with a notable perivascular distribution. CD3 immunohistochemical stain, original magnification<span class="elsevierStyleHsp" style=""></span>×25. B, Cytoplasm positive for CD56 in the perivascular neoplastic lymphocytic infiltrate. CD56 immunohistochemical stain, original magnification<span class="elsevierStyleHsp" style=""></span>×200. C, Cytoplasm positive for the protease granzyme B, present in activated cytotoxic T-cells and in natural killer cells. Granzyme B immunohistochemical stain, original magnification<span class="elsevierStyleHsp" style=""></span>×200. D, Nucleus of the malignant lymphocytes positive for Epstein-Barr virus mRNA. Epstein-Barr-encoded small RNAs (EBER) stain, original magnification<span class="elsevierStyleHsp" style=""></span>×400.</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" => "Nasal natural killer/T cell lymphoma with cutaneous involvement: Case report and Chinese literature review reported in China mainland" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "B. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 11 | 11 | 22 |
| 2024 Octubre | 97 | 52 | 149 |
| 2024 Septiembre | 102 | 31 | 133 |
| 2024 Agosto | 134 | 68 | 202 |
| 2024 Julio | 85 | 43 | 128 |
| 2024 Junio | 93 | 49 | 142 |
| 2024 Mayo | 73 | 43 | 116 |
| 2024 Abril | 92 | 33 | 125 |
| 2024 Marzo | 82 | 35 | 117 |
| 2024 Febrero | 63 | 35 | 98 |
| 2024 Enero | 62 | 29 | 91 |
| 2023 Diciembre | 66 | 14 | 80 |
| 2023 Noviembre | 83 | 23 | 106 |
| 2023 Octubre | 85 | 21 | 106 |
| 2023 Septiembre | 70 | 26 | 96 |
| 2023 Agosto | 51 | 17 | 68 |
| 2023 Julio | 98 | 47 | 145 |
| 2023 Junio | 72 | 22 | 94 |
| 2023 Mayo | 59 | 24 | 83 |
| 2023 Abril | 68 | 20 | 88 |
| 2023 Marzo | 52 | 16 | 68 |
| 2023 Febrero | 63 | 25 | 88 |
| 2023 Enero | 41 | 36 | 77 |
| 2022 Diciembre | 78 | 53 | 131 |
| 2022 Noviembre | 37 | 22 | 59 |
| 2022 Octubre | 31 | 14 | 45 |
| 2022 Septiembre | 26 | 41 | 67 |
| 2022 Agosto | 32 | 37 | 69 |
| 2022 Julio | 27 | 43 | 70 |
| 2022 Junio | 28 | 30 | 58 |
| 2022 Mayo | 85 | 35 | 120 |
| 2022 Abril | 58 | 44 | 102 |
| 2022 Marzo | 71 | 62 | 133 |
| 2022 Febrero | 71 | 38 | 109 |
| 2022 Enero | 81 | 44 | 125 |
| 2021 Diciembre | 54 | 55 | 109 |
| 2021 Noviembre | 68 | 45 | 113 |
| 2021 Octubre | 64 | 51 | 115 |
| 2021 Septiembre | 57 | 40 | 97 |
| 2021 Agosto | 83 | 40 | 123 |
| 2021 Julio | 64 | 35 | 99 |
| 2021 Junio | 76 | 31 | 107 |
| 2021 Mayo | 67 | 53 | 120 |
| 2021 Abril | 159 | 93 | 252 |
| 2021 Marzo | 108 | 40 | 148 |
| 2021 Febrero | 94 | 31 | 125 |
| 2021 Enero | 144 | 15 | 159 |
| 2020 Diciembre | 58 | 25 | 83 |
| 2020 Noviembre | 48 | 19 | 67 |
| 2020 Octubre | 37 | 12 | 49 |
| 2020 Septiembre | 45 | 18 | 63 |
| 2020 Agosto | 28 | 29 | 57 |
| 2020 Julio | 37 | 17 | 54 |
| 2020 Junio | 39 | 29 | 68 |
| 2020 Mayo | 26 | 13 | 39 |
| 2020 Abril | 24 | 13 | 37 |
| 2020 Marzo | 21 | 12 | 33 |
| 2020 Febrero | 2 | 0 | 2 |
| 2020 Enero | 4 | 0 | 4 |
| 2019 Diciembre | 4 | 0 | 4 |
| 2019 Noviembre | 4 | 0 | 4 |
| 2019 Septiembre | 5 | 0 | 5 |
| 2019 Agosto | 6 | 0 | 6 |
| 2019 Julio | 5 | 0 | 5 |
| 2019 Junio | 4 | 0 | 4 |
| 2019 Mayo | 5 | 0 | 5 |
| 2019 Abril | 2 | 0 | 2 |
| 2019 Febrero | 4 | 0 | 4 |
| 2019 Enero | 2 | 0 | 2 |
| 2018 Diciembre | 3 | 0 | 3 |
| 2018 Noviembre | 3 | 0 | 3 |
| 2018 Octubre | 3 | 0 | 3 |
| 2018 Septiembre | 4 | 0 | 4 |
| 2018 Febrero | 21 | 6 | 27 |
| 2018 Enero | 48 | 8 | 56 |
| 2017 Diciembre | 48 | 15 | 63 |
| 2017 Noviembre | 34 | 5 | 39 |
| 2017 Octubre | 40 | 11 | 51 |
| 2017 Septiembre | 193 | 27 | 220 |
| 2017 Agosto | 13 | 11 | 24 |
| 2017 Julio | 6 | 14 | 20 |
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