Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil
IntroductionHuman T-lymphotropic virus type-1 (HTLV-1) is endemic in some countries, including Brazil. HTLV-1 is the etiological agent of adult T-cell leukemia-lymphoma (ATLL), a rare and aggressive CD4+ T-lymphocyte malignancy. ATLL affects 1–5% of virus carriers. Dermatological involvement occurs...
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Frontiers Media S.A.
2025-02-01
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| author | Mariana Valente José Antonio Sanches Youko Nukui Jade Cury-Martins Bruno Castro Souza Juliana Pereira Denis Miyashiro |
| author_facet | Mariana Valente José Antonio Sanches Youko Nukui Jade Cury-Martins Bruno Castro Souza Juliana Pereira Denis Miyashiro |
| author_sort | Mariana Valente |
| collection | DOAJ |
| description | IntroductionHuman T-lymphotropic virus type-1 (HTLV-1) is endemic in some countries, including Brazil. HTLV-1 is the etiological agent of adult T-cell leukemia-lymphoma (ATLL), a rare and aggressive CD4+ T-lymphocyte malignancy. ATLL affects 1–5% of virus carriers. Dermatological involvement occurs in 40–70%. Diagnosis is based on clinicopathologic correlation and HTLV-1 serology. There are few therapeutic options so far.MethodsThis is an observational retrospective cohort study with ATLL patients followed in a tertiary hospital in São Paulo, Brazil. Data were collected at diagnosis. Survival curves using the Kaplan–Meier method were analyzed with log-rank test, univariate and multivariate analyses were performed with the Cox proportional hazards model.ResultsForty-four patients were studied, 24 females (54.5%), and 20 males (45.5%). The median age at diagnosis was 59.4 years. Classification at diagnosis was: 16 (36.4%) chronic (93.7% unfavorable, 6.2% favorable), 14 (31.8%) acute, 10 (22.7%) smoldering, four (9.1%) lymphoma, and none with primary cutaneous tumoral. Regarding skin lesions, 18 (40.9%) had plaques; 15 (34.1%) nodules/tumors; 11 (25.0%) papules; 10 (22.7%) erythroderma; seven (15.9%) patches; two (4.5%) ichthyosis; one (2.3%) purpuric lesions. Epidermotropism/exocytosis of lymphocytes was observed in 25 patients (62.5%), and Pautrier microabscesses in three (7.3%). Four patients (10.0%) had subcutaneous involvement, two (5.0%) folliculotropism, two (5.0%) angiocentrism, and one (2.5%) perineural involvement. Ten patients (25.0%) presented a lichenoid pattern. Thirty-four patients (79.1%) had increased lactate dehydrogenase; 20 (45.5%) lymphocytosis; six (13.6%) flower cells in peripheral blood; six (14.6%) hypercalcemia; five (12.2%) hypoalbuminemia. Beta-2 microglobulin was increased in all 24 cases investigated. Monoclonal T-lymphocytes were observed in the blood of 23 patients (76.7%) and the skin of 19 (76.0%). Thirty patients (68.2%) died. Median overall survival was 32.3 months. Acute and chronic unfavorable forms had worse prognoses, with median overall survival of 23.3 and 34.1 months, respectively (p = 0.0011). After multivariate analysis, Shimoyama classification (acute) and urea levels were associated with poorer prognoses.ConclusionWe described a large Brazilian cohort of ATLL with cutaneous involvement. Description of clinical, pathology, laboratory, and follow-up data, and factors associated with poorer survival is essential to provide better care and to improve the quality of life of these patients. |
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| spelling | doaj-art-59072a8ae6504c54a9e69091e064f3702025-02-07T08:59:53ZengFrontiers Media S.A.Frontiers in Medicine2296-858X2025-02-011210.3389/fmed.2025.15058651505865Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in BrazilMariana Valente0José Antonio Sanches1Youko Nukui2Jade Cury-Martins3Bruno Castro Souza4Juliana Pereira5Denis Miyashiro6Department of Dermatology, University of São Paulo, São Paulo, BrazilDepartment of Dermatology, University of São Paulo, São Paulo, BrazilDiscipline of Hematology, University of São Paulo, São Paulo, BrazilDepartment of Dermatology, University of São Paulo, São Paulo, BrazilUniversity of São Paulo, São Paulo, BrazilDiscipline of Hematology, University of São Paulo, São Paulo, BrazilDepartment of Dermatology, University of São Paulo Medical School, São Paulo, BrazilIntroductionHuman T-lymphotropic virus type-1 (HTLV-1) is endemic in some countries, including Brazil. HTLV-1 is the etiological agent of adult T-cell leukemia-lymphoma (ATLL), a rare and aggressive CD4+ T-lymphocyte malignancy. ATLL affects 1–5% of virus carriers. Dermatological involvement occurs in 40–70%. Diagnosis is based on clinicopathologic correlation and HTLV-1 serology. There are few therapeutic options so far.MethodsThis is an observational retrospective cohort study with ATLL patients followed in a tertiary hospital in São Paulo, Brazil. Data were collected at diagnosis. Survival curves using the Kaplan–Meier method were analyzed with log-rank test, univariate and multivariate analyses were performed with the Cox proportional hazards model.ResultsForty-four patients were studied, 24 females (54.5%), and 20 males (45.5%). The median age at diagnosis was 59.4 years. Classification at diagnosis was: 16 (36.4%) chronic (93.7% unfavorable, 6.2% favorable), 14 (31.8%) acute, 10 (22.7%) smoldering, four (9.1%) lymphoma, and none with primary cutaneous tumoral. Regarding skin lesions, 18 (40.9%) had plaques; 15 (34.1%) nodules/tumors; 11 (25.0%) papules; 10 (22.7%) erythroderma; seven (15.9%) patches; two (4.5%) ichthyosis; one (2.3%) purpuric lesions. Epidermotropism/exocytosis of lymphocytes was observed in 25 patients (62.5%), and Pautrier microabscesses in three (7.3%). Four patients (10.0%) had subcutaneous involvement, two (5.0%) folliculotropism, two (5.0%) angiocentrism, and one (2.5%) perineural involvement. Ten patients (25.0%) presented a lichenoid pattern. Thirty-four patients (79.1%) had increased lactate dehydrogenase; 20 (45.5%) lymphocytosis; six (13.6%) flower cells in peripheral blood; six (14.6%) hypercalcemia; five (12.2%) hypoalbuminemia. Beta-2 microglobulin was increased in all 24 cases investigated. Monoclonal T-lymphocytes were observed in the blood of 23 patients (76.7%) and the skin of 19 (76.0%). Thirty patients (68.2%) died. Median overall survival was 32.3 months. Acute and chronic unfavorable forms had worse prognoses, with median overall survival of 23.3 and 34.1 months, respectively (p = 0.0011). After multivariate analysis, Shimoyama classification (acute) and urea levels were associated with poorer prognoses.ConclusionWe described a large Brazilian cohort of ATLL with cutaneous involvement. Description of clinical, pathology, laboratory, and follow-up data, and factors associated with poorer survival is essential to provide better care and to improve the quality of life of these patients.https://www.frontiersin.org/articles/10.3389/fmed.2025.1505865/fulladult T-cell leukemia-lymphomacutaneous T-cell lymphomaHTLV-1 infectionsHTLV-1cohort |
| spellingShingle | Mariana Valente José Antonio Sanches Youko Nukui Jade Cury-Martins Bruno Castro Souza Juliana Pereira Denis Miyashiro Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil Frontiers in Medicine adult T-cell leukemia-lymphoma cutaneous T-cell lymphoma HTLV-1 infections HTLV-1 cohort |
| title | Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil |
| title_full | Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil |
| title_fullStr | Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil |
| title_full_unstemmed | Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil |
| title_short | Characterization of adult T-cell leukemia/lymphoma patients with specific skin lesions in a tertiary dermatological service in Brazil |
| title_sort | characterization of adult t cell leukemia lymphoma patients with specific skin lesions in a tertiary dermatological service in brazil |
| topic | adult T-cell leukemia-lymphoma cutaneous T-cell lymphoma HTLV-1 infections HTLV-1 cohort |
| url | https://www.frontiersin.org/articles/10.3389/fmed.2025.1505865/full |
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