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      CD30-positive cutaneous lymphoma: report of four cases with an emphasis on clinicopathological correlations*

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          Abstract

          The classification of cutaneous lymphomas is multidisciplinary and requires the correlation between clinical, histopathological, immunohistochemical, and molecular diagnostic elements. In this article, we present four different cases of CD30-positive T-cell lymphoma with cutaneous manifestations. We compare cases with definitive diagnosis of papulosis lymphomatoid type C, primary cutaneous anaplastic large T-cell lymphoma, systemic anaplastic large T-cell lymphoma with secondary skin involvement, and mycosis fungoides with large cell transformation, highlighting the importance of clinicopathological correlation to classify these cases.

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          Most cited references25

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          Brentuximab vedotin.

          Brentuximab vedotin (SGN-35; Adcetris®) is an anti-CD30 antibody conjugated via a protease-cleavable linker to the potent anti-microtubule agent monomethyl auristatin E (MMAE). Following binding to CD30, brentuximab vedotin is rapidly internalized and transported to lysosomes where MMAE is released and binds to tubulin, leading to cell cycle arrest and apoptosis. Several trials have shown durable antitumor activity with a manageable safety profile in patients with relapsed/refractory Hodgkin lymphoma, systemic anaplastic large cell lymphoma, or primary cutaneous CD30-positive lymphoproliferative disorders. Peripheral sensory neuropathy is a significant adverse event associated with brentuximab vedotin administration. Neuropathy symptoms are cumulative and dose-related. Multiple ongoing trials are currently evaluating brentuximab vedotin alone or in combination with other agents in relapsed/refractory patients, as well as patients with newly diagnosed disease.
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            Prognostic factors in transformed mycosis fungoides: a retrospective analysis of 100 cases.

            Large cell transformation (LCT) in mycosis fungoides (MF) is generally associated with an aggressive clinical course and poor survival, requiring aggressive therapeutic approach. However, a proportion of cases may follow an indolent clinical course. To identify prognostic factors, we analyzed the prognostic relevance of clinical, histologic, and immunophenotypical features in a large cohort of transformed MF patients, including 75 patients with only skin lesions, 19 patients with LCT in skin and lymph nodes, and 6 patients with LCT in lymph nodes only. Multivariate analysis of the total group showed that CD30 negativity, folliculotropic MF, extent of skin lesions and extracutaneous transformation were associated with reduced disease-specific survival (DSS) and, except for CD30 negativity and folliculotropic MF, also overall survival. In a multivariate analysis of 75 patients with only skin lesions at the time of LCT, CD30 negativity, folliculotropic MF and extent of skin lesions were independent parameters for both DSS and overall survival. Using the most discriminating parameters as a prognostic index, in both study groups differences in DSS between patients with 0-1 unfavorable prognostic factor(s) and ≥ 2 unfavorable prognostic factors were statistically significant (P < .001). This prognostic index may be helpful in predicting prognosis and selecting the most appropriate treatment in patients with transformed MF.
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              How I treat mycosis fungoides and Sézary syndrome.

              The most common subtypes of primary cutaneous T-cell lymphomas are mycosis fungoides (MF) and Sézary syndrome (SS). The majority of patients have indolent disease; and given the incurable nature of MF/SS, management should focus on improving symptoms and cosmesis while limiting toxicity. Management of MF/SS should use a "stage-based" approach; treatment of early-stage disease (IA-IIA) typically involves skin directed therapies that include topical corticosteroids, phototherapy (psoralen plus ultraviolet A radiation or ultraviolet B radiation), topical chemotherapy, topical or systemic bexarotene, and radiotherapy. Systemic approaches are used for recalcitrant early-stage disease, advanced-stage disease (IIB-IV), and transformed disease and include retinoids, such as bexarotene, interferon-alpha, histone deacetylase inhibitors, the fusion toxin denileukin diftitox, systemic chemotherapy including transplantation, and extracorporeal photopheresis. Examples of drugs under active investigation include new histone deacetylase inhibitors, forodesine, monoclonal antibodies, proteasome inhibitors, and immunomodulatory agents, such as lenalidomide. It is appropriate to consider patients for novel agents within clinical trials if they have failed front-line therapy and before chemotherapy is used.
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                Author and article information

                Journal
                An Bras Dermatol
                An Bras Dermatol
                abd
                Anais Brasileiros de Dermatologia
                Sociedade Brasileira de Dermatologia
                0365-0596
                1806-4841
                Jan-Feb 2017
                Jan-Feb 2017
                : 92
                : 1
                : 86-91
                Affiliations
                [1 ]Hospital Federal de Bonsucesso - Bonsucesso (RJ), Brazil
                [2 ]Instituto Nacional de Câncer (INCA) - Rio de Janeiro (RJ), Brazil
                Author notes
                Mailing address: Samira Barroso Jorge, Av. Londres, 616 - Prédio 6, Bonsucesso, 21041-030 Rio de Janeiro, RJ - Brazil. E-mail: samirabarroso@ 123456yahoo.com.br
                Article
                10.1590/abd1806-4841.20174807
                5312184
                28225962
                942077c8-4b20-4149-9f22-32c9970d7f29
                ©2017 by Anais Brasileiros de Dermatologia

                This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided the original work is properly cited.

                History
                : 05 June 2015
                : 13 October 2015
                Categories
                Dermatopathology

                anaplastic large cell lymphoma,cutaneous t-cell lymphoma,lymphomatoid papulosis,lymphoproliferative disorders,mycosis fungoides,primary cutaneous anaplastic large cell lymphoma

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