Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type

Background: Adult T-cell leukemia/lymphoma (ATLL) is a distinctive peripheral T- lymphocytic malignancy associated with human T-cell lymphotropic virus type 1 (HTLV-1). It may closely resemble other skin lymphomas, particularly mycosis fungoides (MF). Case report: A 38-year-old woman presented s...

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Autores principales:	Julia Ocampo Lyra-da Sylva, Yung Bruno de Mello Gonzaga, Otávio de Melo Espindola, Maria José de Andrada-Serpa, Cassio Dib, Thiago Jeunon
Formato:	article
Lenguaje:	EN
Publicado:	Mattioli1885 2012
Materias:	T-cell leukemia/lymphoma ATLL lymphoma Dermatology RL1-803
Acceso en línea:	https://doaj.org/article/099a0da7c3bf4ac59da7511849570d1b
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spelling	oai:doaj.org-article:099a0da7c3bf4ac59da7511849570d1b2021-11-17T08:33:12ZAdult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type2160-9381https://doaj.org/article/099a0da7c3bf4ac59da7511849570d1b2012-04-01T00:00:00Zhttp://dpcj.org/index.php/dpc/article/view/990https://doaj.org/toc/2160-9381 Background: Adult T-cell leukemia/lymphoma (ATLL) is a distinctive peripheral T- lymphocytic malignancy associated with human T-cell lymphotropic virus type 1 (HTLV-1). It may closely resemble other skin lymphomas, particularly mycosis fungoides (MF). Case report: A 38-year-old woman presented some ellipsoid scaling patches lasting 18 months and developed a large tumoral lesion in the abdomen, which were previously diagnosed as MF. Although histopathologic and immunohistochemistry findings were in consonance with this diagnosis, the fast progression of the disease raised the suspicion that it could represent another type of T-cell lymphoma. The work-up revealed a positive anti-HTLV-1 serology and molecular studies confirmed the monoclonal integration of HTLV-1 provirus into neoplastic cells of the skin, but not into circulating lymphocytes. Extensive investigations were unable to demonstrate any systemic involvement. The final diagnosis was of primary cutaneous type of ATLL. The patient was submitted to a chemotherapy regimen with cyclophosphamide, doxorubicin, vincristine and prednisone, later to conjugated dexamethasone and surgical cytoreduction and then to a second line treatment with gemcitabine, resulting in partial response. A bone marrow heterologous transplantation was performed, but failed to achieve a sustained remission. Discussion: ATLL is a rare lymphoid malignancy in non-endemic HTLV-1 areas, the diagnosis of which could be missed if not highly suspected. In addition to the four subtypes of Shimoyama classification (acute, lymphomatous, chronic and smoldering), a fifth one denominated primary cutaneous and characterized by presence of lesions only in the skin had been proposed and is herein exemplified. Julia Ocampo Lyra-da SylvaYung Bruno de Mello GonzagaOtávio de Melo EspindolaMaria José de Andrada-SerpaCassio DibThiago JeunonMattioli1885articleT-cell leukemia/lymphomaATLLlymphomaDermatologyRL1-803ENDermatology Practical & Conceptual (2012)
institution	DOAJ
collection	DOAJ
language	EN
topic	T-cell leukemia/lymphoma ATLL lymphoma Dermatology RL1-803
spellingShingle	T-cell leukemia/lymphoma ATLL lymphoma Dermatology RL1-803 Julia Ocampo Lyra-da Sylva Yung Bruno de Mello Gonzaga Otávio de Melo Espindola Maria José de Andrada-Serpa Cassio Dib Thiago Jeunon Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
description	Background: Adult T-cell leukemia/lymphoma (ATLL) is a distinctive peripheral T- lymphocytic malignancy associated with human T-cell lymphotropic virus type 1 (HTLV-1). It may closely resemble other skin lymphomas, particularly mycosis fungoides (MF). Case report: A 38-year-old woman presented some ellipsoid scaling patches lasting 18 months and developed a large tumoral lesion in the abdomen, which were previously diagnosed as MF. Although histopathologic and immunohistochemistry findings were in consonance with this diagnosis, the fast progression of the disease raised the suspicion that it could represent another type of T-cell lymphoma. The work-up revealed a positive anti-HTLV-1 serology and molecular studies confirmed the monoclonal integration of HTLV-1 provirus into neoplastic cells of the skin, but not into circulating lymphocytes. Extensive investigations were unable to demonstrate any systemic involvement. The final diagnosis was of primary cutaneous type of ATLL. The patient was submitted to a chemotherapy regimen with cyclophosphamide, doxorubicin, vincristine and prednisone, later to conjugated dexamethasone and surgical cytoreduction and then to a second line treatment with gemcitabine, resulting in partial response. A bone marrow heterologous transplantation was performed, but failed to achieve a sustained remission. Discussion: ATLL is a rare lymphoid malignancy in non-endemic HTLV-1 areas, the diagnosis of which could be missed if not highly suspected. In addition to the four subtypes of Shimoyama classification (acute, lymphomatous, chronic and smoldering), a fifth one denominated primary cutaneous and characterized by presence of lesions only in the skin had been proposed and is herein exemplified.
format	article
author	Julia Ocampo Lyra-da Sylva Yung Bruno de Mello Gonzaga Otávio de Melo Espindola Maria José de Andrada-Serpa Cassio Dib Thiago Jeunon
author_facet	Julia Ocampo Lyra-da Sylva Yung Bruno de Mello Gonzaga Otávio de Melo Espindola Maria José de Andrada-Serpa Cassio Dib Thiago Jeunon
author_sort	Julia Ocampo Lyra-da Sylva
title	Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
title_short	Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
title_full	Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
title_fullStr	Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
title_full_unstemmed	Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
title_sort	adult t-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type
publisher	Mattioli1885
publishDate	2012
url	https://doaj.org/article/099a0da7c3bf4ac59da7511849570d1b
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Adult T-cell leukemia/lymphoma: a case report of primary cutaneous tumoral type

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