Abstract
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.
Highlights
Lymphomas may involve the skin primarily, when there is no extracutaneous disease by the time of the diagnosis, or secondarily, as an infiltration of a lymphoma from another site, most often lymph nodes [1,2]
Adult T-cell leukemia/lymphoma (ATLL) is a distinctive peripheral T- lymphocytic malignancy associated with human T-cell lymphotropic virus type 1 (HTLV-1)
Adult T-cell leukemia/lymphoma (ATLL) is a lymphoid malignancy etiologically related to human T-cell lymphotropic virus type 1 (HTLV-1) infection
Summary
Lymphomas may involve the skin primarily, when there is no extracutaneous disease by the time of the diagnosis, or secondarily, as an infiltration of a lymphoma from another site, most often lymph nodes [1,2]. Mycosis fungoides (MF) is the most common primary cutaneous lymphoma, accounting for almost 50% of cases of lymphoma restricted to the skin It is characterized by an indolent course, evolving from patches to plaques and rarely eventuating in tumors over the course of years or even decades [3,4]. Adult T-cell leukemia/lymphoma (ATLL) is a lymphoid malignancy etiologically related to human T-cell lymphotropic virus type 1 (HTLV-1) infection. It is frequently a multi- systemic disease and has been classically classified by Shimoyama et al into four subtypes, namely acute, lymphomatous, chronic and smoldering [5].
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