The role of 9-O-acetylated ganglioside D3 (CD60) and 4 1 (CD49d) expression in predicting the survival of patients with Sezary syndrome
| العنوان: | The role of 9-O-acetylated ganglioside D3 (CD60) and 4 1 (CD49d) expression in predicting the survival of patients with Sezary syndrome |
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| المؤلفون: | Damiano Abeni, Maria Grazia Narducci, Maria Picchio, Giandomenico Russo, Elisabetta Caprini, Adriano Mari, D. Pomponi, Maria Antonietta Pilla, Marina Frontani, Enrico Scala, Giuseppe Alfonso Lombardo |
| المصدر: | Haematologica. 95:1905-1912 |
| بيانات النشر: | Ferrata Storti Foundation (Haematologica), 2010. |
| سنة النشر: | 2010 |
| مصطلحات موضوعية: | Adult, CD4-Positive T-Lymphocytes, Male, medicine.medical_specialty, Skin Neoplasms, Dipeptidyl Peptidase 4, Integrin alpha4, Receptors, Antigen, T-Cell, alpha-beta, Population, Antigens, CD7, Disease-Free Survival, Gangliosides, White blood cell, Internal medicine, Biomarkers, Tumor, Humans, Sezary Syndrome, Medicine, education, Survival rate, Aged, education.field_of_study, Mycosis fungoides, Hematology, business.industry, Cutaneous T-cell lymphoma, Middle Aged, Flow Cytometry, medicine.disease, Peripheral T-cell lymphoma, Lymphoma, Gene Expression Regulation, Neoplastic, Survival Rate, medicine.anatomical_structure, Immunology, Original Article, Female, business |
| الوصف: | Sézary syndrome is a rare and very aggressive leukemic variant of cutaneous T-cell lymphoma characterized by extensive skin involvement and a malignant circulating CD4(+) T-cell clone which homes to the skin, over-expresses CD60, and lacks CD7, CD26 and CD49d. So far prognostic markers in this disease are limited to treatment with systemic steroids, age, serum lactate dehydrogenase, and a white blood cell count of 20×10(9)/L or higher: no other biological marker with prognostic value, especially related to malignant cells, has been described.We used flow activated cell sorting analysis to compare the distribution of the T-cell receptor-Vβ repertoire and several surface molecules (CD7, CD26, CD49d and CD60) within the circulating CD4(+) T-cell population in 62 patients with Sézary syndrome, 180 with mycosis fungoides, 6 with B-cell lymphomas, and 19 with chronic eczema. We calculated the 5-year overall survival of patients with Sézary syndrome after first hospital admission using Kaplan-Meier product-limit estimates and hazard ratios from the Cox proportional hazards model.We found that both higher number of CD60(+) and lower number of CD49d(+) cells within circulating CD4(+) T cells at disease presentation were significantly associated with a lower probability of survival. An exceedingly high risk of death was observed for patients with a combination of a high proportion of CD4(+)CD60(+) cells (≥ 0.5×10(9)/L) and low proportion of CD4(+)CD49d(+) cells (0.5×10(9)/L) (hazard ratio = 12.303, 95% confidence interval 1.5-95.9; P0.02). In addition, a skewed usage of T-cell receptor-Vβ subfamilies was observed in the circulating T-cell clone for 61.9% of all patients with Sézary syndrome, T-cell receptor-Vβ 2 and 5.1 subfamilies being the most frequently represented (42.8%), followed by T-cell receptor-Vβ 12 and 13.1.In this study we showed that up-regulation of CD60 and down-regulation of CD49d on circulating CD4(+) T cells are two useful markers for predicting a very poor outcome in patients with Sézary syndrome. |
| تدمد: | 1592-8721 0390-6078 |
| الوصول الحر: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::09ca245471d262d4241f26b02d54cd8cTest https://doi.org/10.3324/haematol.2010.026260Test |
| حقوق: | OPEN |
| رقم الانضمام: | edsair.doi.dedup.....09ca245471d262d4241f26b02d54cd8c |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15928721 03906078 |
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