Case and Research Letters
Infiltración neoplásica cutánea por un linfoma linfoplasmocítico en una paciente con macroglobulinemia de Waldenström
Neoplastic Cutaneous Infiltration by Lymphoplasmacytic Lymphoma in a Patient with Waldenström’s Macroglobulinemia
Margarida Moura Valejo Coelho
, Alexandre João, Filipa Rocha Páris
Corresponding author
Servicio de Dermatología y Venereología, Centro Hospitalar Universitário de Lisboa Central, Alameda de Santo António dos Capuchos, 1169-050 Lisbon, Portugal
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A nivel dérmico y subcutáneo se observaba la presencia de una infiltración densa y difusa, compuesta por linfocitos pequeños, células linfoplasmocitoides y células plasmáticas (a), resultando además las tinciones positivas para el CD20 (b), el CD138 (c) y la IgM (d), limitada a la cadena ligera kappa (e,f). Estos datos en su conjunto confirmaron la afectación cutánea neoplásica por una macroglobulinemia de Waldenström.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "M.M. Valejo Coelho, A. João, F. Rocha Páris" "autores" => array:3 [ 0 => array:2 [ "nombre" => "M.M." "apellidos" => "Valejo Coelho" ] 1 => array:2 [ "nombre" => "A." "apellidos" => "João" ] 2 => array:2 [ "nombre" => "F." 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The patient was referred to our Dermatology Department due to the recent occurrence of ill-defined, erythematous, infiltrated patches on the extensor surface of both arms, painful upon palpation (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). She had no history of previous dermatological disorders and no triggering factors were identified. Cachexia and hepatosplenomegaly were also apparent, but the rest of the physical examination was unremarkable. Laboratory results showed a normocytic, normochromic anemia (hemoglobin 9.1 × 10 g/L), hypoalbuminemia (25.8 g/L), high levels of serum immunoglobulin M (IgM; 70.50 g/L) and of several tumor markers, namely cancer antigen (CA) 125, CA 19−9, CA 72−4 and carcinoembryonic antigen (CEA).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0010" class="elsevierStylePara elsevierViewall">As the clinical findings were non-specific, we performed a deep skin biopsy of one lesion. The histopathological examination showed a diffuse, dense dermal and subcutaneous infiltration of small lymphocytes, lymphoplasmacytoid cells and plasma cells, staining positive for CD20, CD79a, CD138 and IgM, with <span class="elsevierStyleItalic">kappa</span> light chain restriction (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>), and negative for several T-cell markers, namely CD3, CD4 and CD8. These findings confirmed the diagnosis of specific skin infiltration by WM (lymphoplasmacytic lymphoma). Another scheme of systemic therapy directed to WM (bortezomib and dexamethasone) was started, together with topical clobetasol propionate on the skin lesions for local symptomatic relief, without significant clinical improvement, however. The progression of two concurrent advanced oncological diseases – colon cancer and WM – led to a rapidly progressive clinical deterioration that culminated in the patient’s death few months later.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">WM is a lymphoproliferative disease, characterized by clonal proliferation of lymphoplasmacytoid cells producing a monoclonal IgM protein.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> A broad spectrum of cutaneous disorders has been associated with this monoclonal gammopathy, including non-neoplastic and neoplastic skin manifestations,<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a> which occur in only approximately 5% of the patients with WM.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> These can be due to several pathogenic mechanisms, namely: 1) specific cutaneous infiltration by neoplastic cells or deposition of their cellular products, particularly monoclonal IgM (“macroglobulinemia cutis”); 2) secondary to paraproteinemia, including mucocutaneous manifestations of hyperviscosity, cryoglobulinemia or autoimmune phenomena; 3) miscellaneous manifestations of uncertain etiology, as urticaria and amyloid light-chain (AL) amyloidosis.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–3</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">Specific infiltration of the skin by proliferating lymphoplasmacytoid neoplastic cells is, in fact, the rarest cutaneous manifestation of WM, with only few similar cases having been reported to date, but it should be considered in the differential diagnosis of asymptomatic or symptomatic, infiltrated skin lesions in these patients.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–6</span></a> Histopathological examination of skin specimens with immunoperoxidase stains is key in establishing the correct diagnosis.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> These lesions result from skin infiltration by malignant cells, and treatment should therefore target the underlying hematological malignancy,<span class="elsevierStyleSup">1</span> although clinical responses have been inconsistent.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Interestingly, the presence of cutaneous involvement does not appear to be clearly related with disease course and progression, according to the available literature.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Nevertheless, further research is warranted.</p><p id="par0025" class="elsevierStylePara elsevierViewall">We report this clinical case to draw attention to an uncommon dermatological expression of a systemic hematologic malignancy and the importance of considering such possibility in appropriate clinical settings.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2018-04-08" "fechaAceptado" => "2018-09-19" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Valejo Coelho M.M., João A., Rocha Páris F. Infiltración neoplásica cutánea por un linfoma linfoplasmocítico en una paciente con macroglobulinemia de Waldenström. Actas Dermosifiliogr. 2020;111:528–531.</p>" ] ] "multimedia" => array:2 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1133 "Ancho" => 1500 "Tamanyo" => 215408 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Clinical presentation: Ill-defined, erythematous, infiltrated patches on the extensor surface of the patient’s right (a) and left (b) arms, which were painful upon palpation.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Fig. 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 2829 "Ancho" => 2500 "Tamanyo" => 1754179 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Fig. " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Histopathological findings in skin lesion biopsy: Presence of a diffuse, dense dermal and subcutaneous infiltration (a) by small lymphocytes, lymphoplasmacytoid cells, and plasma cells, staining positive for CD20 (b), CD138 (c) and IgM (d), with <span class="elsevierStyleItalic">kappa</span> light chain restriction (e, f), confirming neoplastic cutaneous involvement by Waldenström’s macroglobulinemia.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Monoclonal gammopathies and associated skin disorders" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "M.S. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 5 | 2 | 7 |
| 2024 October | 66 | 43 | 109 |
| 2024 September | 80 | 23 | 103 |
| 2024 August | 97 | 55 | 152 |
| 2024 July | 68 | 36 | 104 |
| 2024 June | 76 | 47 | 123 |
| 2024 May | 69 | 40 | 109 |
| 2024 April | 79 | 29 | 108 |
| 2024 March | 69 | 22 | 91 |
| 2024 February | 80 | 29 | 109 |
| 2024 January | 72 | 37 | 109 |
| 2023 December | 43 | 13 | 56 |
| 2023 November | 68 | 21 | 89 |
| 2023 October | 71 | 16 | 87 |
| 2023 September | 63 | 30 | 93 |
| 2023 August | 54 | 18 | 72 |
| 2023 July | 54 | 29 | 83 |
| 2023 June | 55 | 22 | 77 |
| 2023 May | 75 | 17 | 92 |
| 2023 April | 34 | 14 | 48 |
| 2023 March | 51 | 26 | 77 |
| 2023 February | 76 | 13 | 89 |
| 2023 January | 42 | 17 | 59 |
| 2022 December | 57 | 30 | 87 |
| 2022 November | 19 | 28 | 47 |
| 2022 October | 24 | 17 | 41 |
| 2022 September | 20 | 28 | 48 |
| 2022 August | 35 | 28 | 63 |
| 2022 July | 23 | 22 | 45 |
| 2022 June | 35 | 23 | 58 |
| 2022 May | 31 | 26 | 57 |
| 2022 April | 35 | 29 | 64 |
| 2022 March | 40 | 44 | 84 |
| 2022 February | 31 | 23 | 54 |
| 2022 January | 36 | 27 | 63 |
| 2021 December | 30 | 39 | 69 |
| 2021 November | 42 | 38 | 80 |
| 2021 October | 35 | 55 | 90 |
| 2021 September | 31 | 42 | 73 |
| 2021 August | 42 | 31 | 73 |
| 2021 July | 27 | 24 | 51 |
| 2021 June | 26 | 15 | 41 |
| 2021 May | 28 | 43 | 71 |
| 2021 April | 95 | 74 | 169 |
| 2021 March | 44 | 26 | 70 |
| 2021 February | 45 | 25 | 70 |
| 2021 January | 27 | 19 | 46 |
| 2020 December | 42 | 14 | 56 |
| 2020 November | 20 | 18 | 38 |
| 2020 October | 38 | 14 | 52 |
| 2020 September | 41 | 32 | 73 |
| 2020 August | 56 | 22 | 78 |
| 2020 July | 23 | 24 | 47 |
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