Case and Research Letters
Primary Cutaneous CD4+ Small/Medium-Sized T-Cell Lymphoma With Spontaneous Regression After Biopsy
Linfoma primario cutáneo T CD4 positivo de linfocitos de pequeño y mediano tamaño con regresión espontánea tras la biopsia
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González Fernández, F. Valdés Pineda, S. Gómez Díez, B. Vivanco Allende" "autores" => array:4 [ 0 => array:4 [ "nombre" => "D." "apellidos" => "González Fernández" "email" => array:1 [ 0 => "danigf81@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" => "F." "apellidos" => "Valdés Pineda" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "S." "apellidos" => "Gómez Díez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 3 => array:3 [ "nombre" => "B." "apellidos" => "Vivanco Allende" "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 Central de Asturias, Oviedo, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Central de Asturias, Oviedo, 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 primario cutáneo T CD4 positivo de linfocitos de pequeño y mediano tamaño con regresión espontánea tras la biopsia" ] ] "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" => 677 "Ancho" => 900 "Tamanyo" => 116958 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A single nodular lesion of 2.5<span class="elsevierStyleHsp" style=""></span>cm diameter on the nasal ala.</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Primary cutaneous CD4+ small/medium-sized T-cell lymphoma (PCSM-TCL) is a rare provisional entity included in the 2005 cutaneous lymphoma classification of the World Health Organization/European Organisation for Research and Treatment of Cancer (WHO/EORTC) in the group of primary cutaneous T-cell lymphomas.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Our patient was a 44-year-old man with no past history of interest. He was seen for a nodular lesion that had appeared on the left nasal ala 3 weeks earlier and had grown progressively. At the time of consultation, this well-defined, indurated nodular lesion measured 2.5<span class="elsevierStyleHsp" style=""></span>cm in diameter and there were no changes in the overlying skin (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No other cutaneous lesions were observed and there were no palpable lymph nodes or organomegaly.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">An incisional biopsy revealed a dense, diffuse lymphocytic infiltrate in the dermis. The lymphocyte population was formed of small-sized lymphocytes associated with another population of medium-sized lymphocytes, both populations showing pleomorphism. No discernible germinal centers were found. Epidermotropism was minimal and there was slight infiltration of the follicular epithelium. Immunohistochemistry revealed a lymphocyte population formed predominantly of T cells that expressed CD3 and CD5. The CD4/CD8 ratio was greater than 2 (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). Analysis of T-cell receptor rearrangement revealed a monoclonal T-cell population.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Blood tests including complete blood count, routine biochemistry, and lactate dehydrogenase were normal, as was cervical-thoracic-abdominal computed tomography, excluding the involvement of other organs. Based on these findings we made a diagnosis of PCSM-TCL.</p><p id="par0025" class="elsevierStylePara elsevierViewall">Since the first follow-up visit, 10 days after the biopsy, the lesion was seen to regress and it had completely disappeared after 3 months (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). In view of the good clinical course, we decided to maintain a wait-and-see approach with frequent follow-up visits. After a year the patient remained asymptomatic and no new lesions had appeared.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">PCSM-TCL is included as a provisional entity in the group of primary cutaneous T-cell lymphomas, both in the 2005 WHO/EORTC classification of cutaneous lymphomas and in the latest (2008) classification of tumors of hematopoietic and lymphoid tissue.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1,2</span></a> It is a rare disease that accounts for approximately 2% to 3% of primary cutaneous lymphomas.<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">1</span></a> It affects men and women equally, and the mean age at the time of diagnosis is 50-60 years, though it can arise at any age—published cases range from 3 to 90 years of age.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Clinical presentation is usually as a solitary, asymptomatic, rapidly growing plaque or mass of up to 3<span class="elsevierStyleHsp" style=""></span>cm diameter; lesions most commonly arise on the face or neck, or on the upper part of the trunk. The surface is typically smooth and may have an erythematous or violaceous appearance.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> There are 2 other forms of presentation with a poorer prognosis, one as an aggressive tumor and the other as multiple lesions with abundant eosinophils observed in the skin biopsy.<a class="elsevierStyleCrossRefs" href="#bib0070"><span class="elsevierStyleSup">4,5</span></a> The histology of the lesions contrasts with the benign course of PCSM-TCL. Dense, nodular or diffuse infiltrates of atypical, pleomorphic small- and medium-sized lymphocytes are found in the superficial and deep dermis and tend to infiltrate the subcutaneous cellular tissue. Although large pleomorphic cells may be observed, by definition these make up less than 30% of the infiltrate. An inflammatory infiltrate of reactive CD8+ T cells is usually present, as well as a variable number of B cells, plasma cells, macrophages, and eosinophils.<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">6</span></a> It is not uncommon to find focal areas of epidermotropism and invasion of adnexal structures. The atypical cells show the following immunohistochemical profile: CD3+, CD4+, CD8–, and CD30–. The proliferation index is less than 30%, and molecular analysis reveals monoclonal rearrangement of the T-cell receptor gene, either of the gamma chain or of the beta chain, in 60% of cases.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–3</span></a> In the differential diagnosis, there are no clear criteria to differentiate PCSM-TCL from benign lymphoid diseases, and the nuclear pleomorphism, immunohistochemistry, and detection of clonality are therefore used to orient the diagnosis to PCSM-TCL.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">3</span></a> Five-year survival is over 90% when presentation is with a single lesion with typical characteristics. This prognosis worsens when presentation is multifocal, and overall 5-year survival is of 60% to 80%.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">1–3</span></a> Poor prognostic factors include CD2–, rapidly growing tumors over 5<span class="elsevierStyleHsp" style=""></span>cm in diameter, the presence of a limited number of accompanying CD8+ T<span class="elsevierStyleHsp" style=""></span>cells and a high ki-67 index.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">3,7</span></a> To date, 3 other cases of spontaneous regression after biopsy have been reported.<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">7–9</span></a> The therapeutic options described in the consensus statement on the treatment of cutaneous lymphomas<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">10</span></a> suggest non-aggressive management for patients with primary or recurrent, single or multiple localized nodular forms, varying from follow-up of the patient to topical or intralesional corticosteroids or even surgery or radiation therapy. Treatment for aggressive forms must be individualized according to the characteristics of the patient.</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: González Fernández D, Valdés Pineda F, Gómez Díez S, Vivanco Allende B. Linfoma primario cutáneo T CD4 positivo de linfocitos de pequeño y mediano tamaño con regresión espontánea tras la biopsia. Actas Dermosifiliogr. 2015;106:767–768.</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" => 677 "Ancho" => 900 "Tamanyo" => 116958 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A single nodular lesion of 2.5<span class="elsevierStyleHsp" style=""></span>cm diameter on the nasal ala.</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" => 363 "Ancho" => 1948 "Tamanyo" => 279668 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">A and B, Biopsy showing the dense lymphocytic infiltrate. Hematoxylin-eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>2 (A) and ×<span class="elsevierStyleHsp" style=""></span>10 (B). C, Detail of the composition of the infiltrate, showing cellular pleomorphism. Hematoxylin–eosin, original magnification ×<span class="elsevierStyleHsp" style=""></span>40. D, Immunohistochemical stain for CD4, original magnification ×<span class="elsevierStyleHsp" style=""></span>2.</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" => 900 "Ancho" => 932 "Tamanyo" => 142556 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Complete regression of the lesion at 3 months.</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" => "WHO-EORTC classification for cutaneous lymphomas" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "R. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 7 | 10 | 17 |
| 2024 Octubre | 72 | 39 | 111 |
| 2024 Septiembre | 78 | 31 | 109 |
| 2024 Agosto | 107 | 66 | 173 |
| 2024 Julio | 74 | 46 | 120 |
| 2024 Junio | 74 | 41 | 115 |
| 2024 Mayo | 64 | 33 | 97 |
| 2024 Abril | 88 | 26 | 114 |
| 2024 Marzo | 85 | 34 | 119 |
| 2024 Febrero | 68 | 27 | 95 |
| 2024 Enero | 61 | 37 | 98 |
| 2023 Diciembre | 49 | 31 | 80 |
| 2023 Noviembre | 59 | 27 | 86 |
| 2023 Octubre | 57 | 18 | 75 |
| 2023 Septiembre | 74 | 31 | 105 |
| 2023 Agosto | 59 | 17 | 76 |
| 2023 Julio | 55 | 35 | 90 |
| 2023 Junio | 56 | 26 | 82 |
| 2023 Mayo | 81 | 30 | 111 |
| 2023 Abril | 53 | 22 | 75 |
| 2023 Marzo | 52 | 32 | 84 |
| 2023 Febrero | 84 | 22 | 106 |
| 2023 Enero | 59 | 36 | 95 |
| 2022 Diciembre | 75 | 57 | 132 |
| 2022 Noviembre | 40 | 31 | 71 |
| 2022 Octubre | 41 | 55 | 96 |
| 2022 Septiembre | 33 | 48 | 81 |
| 2022 Agosto | 53 | 36 | 89 |
| 2022 Julio | 34 | 43 | 77 |
| 2022 Junio | 38 | 29 | 67 |
| 2022 Mayo | 71 | 35 | 106 |
| 2022 Abril | 85 | 36 | 121 |
| 2022 Marzo | 101 | 52 | 153 |
| 2022 Febrero | 94 | 28 | 122 |
| 2022 Enero | 75 | 38 | 113 |
| 2021 Diciembre | 51 | 41 | 92 |
| 2021 Noviembre | 67 | 50 | 117 |
| 2021 Octubre | 48 | 69 | 117 |
| 2021 Septiembre | 53 | 42 | 95 |
| 2021 Agosto | 35 | 47 | 82 |
| 2021 Julio | 44 | 29 | 73 |
| 2021 Junio | 47 | 25 | 72 |
| 2021 Mayo | 28 | 31 | 59 |
| 2021 Abril | 83 | 61 | 144 |
| 2021 Marzo | 88 | 40 | 128 |
| 2021 Febrero | 58 | 36 | 94 |
| 2021 Enero | 48 | 34 | 82 |
| 2020 Diciembre | 51 | 19 | 70 |
| 2020 Noviembre | 34 | 27 | 61 |
| 2020 Octubre | 37 | 20 | 57 |
| 2020 Septiembre | 47 | 30 | 77 |
| 2020 Agosto | 36 | 15 | 51 |
| 2020 Julio | 36 | 23 | 59 |
| 2020 Junio | 38 | 28 | 66 |
| 2020 Mayo | 49 | 29 | 78 |
| 2020 Abril | 36 | 26 | 62 |
| 2020 Marzo | 41 | 23 | 64 |
| 2020 Febrero | 6 | 2 | 8 |
| 2020 Enero | 0 | 1 | 1 |
| 2019 Diciembre | 4 | 2 | 6 |
| 2019 Noviembre | 0 | 5 | 5 |
| 2019 Octubre | 0 | 2 | 2 |
| 2019 Septiembre | 4 | 3 | 7 |
| 2019 Julio | 0 | 9 | 9 |
| 2019 Junio | 2 | 14 | 16 |
| 2019 Mayo | 0 | 37 | 37 |
| 2019 Abril | 0 | 5 | 5 |
| 2019 Marzo | 2 | 7 | 9 |
| 2019 Enero | 6 | 0 | 6 |
| 2018 Noviembre | 1 | 0 | 1 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 3 | 2 | 5 |
| 2018 Agosto | 0 | 1 | 1 |
| 2018 Julio | 0 | 1 | 1 |
| 2018 Junio | 0 | 3 | 3 |
| 2018 Mayo | 0 | 10 | 10 |
| 2018 Marzo | 0 | 11 | 11 |
| 2018 Febrero | 26 | 8 | 34 |
| 2018 Enero | 29 | 7 | 36 |
| 2017 Diciembre | 48 | 13 | 61 |
| 2017 Noviembre | 37 | 10 | 47 |
| 2017 Octubre | 16 | 10 | 26 |
| 2017 Septiembre | 22 | 10 | 32 |
| 2017 Agosto | 25 | 9 | 34 |
| 2017 Julio | 26 | 20 | 46 |
| 2017 Junio | 24 | 21 | 45 |
| 2017 Mayo | 25 | 24 | 49 |
| 2017 Abril | 18 | 9 | 27 |
| 2017 Marzo | 12 | 24 | 36 |
| 2017 Febrero | 19 | 20 | 39 |
| 2017 Enero | 14 | 12 | 26 |
| 2016 Diciembre | 16 | 18 | 34 |
| 2016 Noviembre | 20 | 21 | 41 |
| 2016 Octubre | 16 | 20 | 36 |
| 2016 Septiembre | 0 | 1 | 1 |
| 2016 Agosto | 1 | 3 | 4 |
| 2016 Julio | 1 | 2 | 3 |
| 2016 Junio | 3 | 0 | 3 |
| 2016 Mayo | 1 | 3 | 4 |
| 2016 Enero | 0 | 1 | 1 |
| 2015 Diciembre | 0 | 1 | 1 |
| 2015 Noviembre | 0 | 6 | 6 |
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