Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases...Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases, is even the only m anifestation. Here we report a rare case of ‘cutaneous’adult T-cell leukaemia /lymphoma with neither atypical cells in the peripheral blood nor lymph node inv olvement. All nodular lesions were completely eliminated after local electron be am irradiation (20 Gy/nodule in total). To evaluate whether or not there were re sidual lymphoma cells in the skin, we performed PCR to detect clonal T cell rece ptor γgene rearrangements. The sample from the nodule before irradiation showed evidence of a rearranged band, which was not detected at the same site after tr eatment nor in any peripheral blood. The findings suggest that this procedure is useful for the evaluation of therapeutic effects and the early detection of lym phoma recurrence.展开更多
Background: A dominant T- cell clone can be detected by polymerase chain reaction (PCR) in 40- 90% of cutaneous samples from patients with cutaneous T- cell lymphoma (CTCL). Materials and methods: From 1996 to 2003 we...Background: A dominant T- cell clone can be detected by polymerase chain reaction (PCR) in 40- 90% of cutaneous samples from patients with cutaneous T- cell lymphoma (CTCL). Materials and methods: From 1996 to 2003 we analysed 547 cutaneous biopsies performed to exclude CTCL (mycosis fungoides, MF/Sé zary syndrome, SS). The final diagnosis was benign inflammatory disease (BID) in 353 samples (64.5% )- and CTCL in 194 (35.5% ). T- cell receptor (TCR)- γ gene rearrangement was studied by using a multiplex PCR/heteroduplex (HD) analysis. The PCR results were correlated with the clinical picture, the histological pattern and the presence of T- cell lineage antigen loss, using univariate and multivariate logistic regression analyses. Objective: To determine the sensitivity and specificity of the multiplex PCR/HD analysis and to identify which are the clinical, histopathological or immunophenotypical features significantly associated with a positive T- cell clonality. Results: A clonality was demonstrated in 83.5% of CTCL and in 2.3% of BID (P < 0.001). A significantly higher percentage of clonal cases was associated with the cutaneous T- score (71.4% in T1, 76.1% in T2 and 100% in nodular and erythrodermic MF samples) and with the presence of a T- cell lineage antigen loss (93.9% vs. 77.4% ). Moreover, clonality was closely related to an increase in the histopathological score (51.3% in the samples with a score < 5, compared with 92% in the lesions with ≥ 5). No significant difference in the percentage of clonal cases was found between T1/T2 and T3/T4 lesions with a histopathological score ≥ 5. The multivariate logistic regression showed that the density and extent of the cell infiltrate, the degree of epidermotropism and the presence of cytological atypia share an independent predictive value for clonality in T1/T2 samples, even if the highest odds ratios (3.6) were associated with the density of the cell infiltrate. The disease course of T1/T2 patients was analysed according to the PCR findings. All the PCR- negative patients showed a long- standing stable disease course; on the other hand, a disease progression occurred in 12/87 (13.8% ) positive patients. Conclusions: The multiplex PCR/HD analysis is associated with a high diagnostic accuracy (92.7% ) in CTCL patients. The finding of a clonal T- cell rearrangement is more closely associated with the histological pattern (in particular with the density and extent of the cell infiltrate) rather than with the MF cutaneous T- score or immunophenotype.展开更多
文摘Adult T-cell leukaemia/lymphoma is alymphopro life rativedis ord eraetiologi call yassociated with human T-cell lymphotropic virus type I infection. A cutan eous lesion often develops in the disease, and in rare cases, is even the only m anifestation. Here we report a rare case of ‘cutaneous’adult T-cell leukaemia /lymphoma with neither atypical cells in the peripheral blood nor lymph node inv olvement. All nodular lesions were completely eliminated after local electron be am irradiation (20 Gy/nodule in total). To evaluate whether or not there were re sidual lymphoma cells in the skin, we performed PCR to detect clonal T cell rece ptor γgene rearrangements. The sample from the nodule before irradiation showed evidence of a rearranged band, which was not detected at the same site after tr eatment nor in any peripheral blood. The findings suggest that this procedure is useful for the evaluation of therapeutic effects and the early detection of lym phoma recurrence.
文摘Background: A dominant T- cell clone can be detected by polymerase chain reaction (PCR) in 40- 90% of cutaneous samples from patients with cutaneous T- cell lymphoma (CTCL). Materials and methods: From 1996 to 2003 we analysed 547 cutaneous biopsies performed to exclude CTCL (mycosis fungoides, MF/Sé zary syndrome, SS). The final diagnosis was benign inflammatory disease (BID) in 353 samples (64.5% )- and CTCL in 194 (35.5% ). T- cell receptor (TCR)- γ gene rearrangement was studied by using a multiplex PCR/heteroduplex (HD) analysis. The PCR results were correlated with the clinical picture, the histological pattern and the presence of T- cell lineage antigen loss, using univariate and multivariate logistic regression analyses. Objective: To determine the sensitivity and specificity of the multiplex PCR/HD analysis and to identify which are the clinical, histopathological or immunophenotypical features significantly associated with a positive T- cell clonality. Results: A clonality was demonstrated in 83.5% of CTCL and in 2.3% of BID (P < 0.001). A significantly higher percentage of clonal cases was associated with the cutaneous T- score (71.4% in T1, 76.1% in T2 and 100% in nodular and erythrodermic MF samples) and with the presence of a T- cell lineage antigen loss (93.9% vs. 77.4% ). Moreover, clonality was closely related to an increase in the histopathological score (51.3% in the samples with a score < 5, compared with 92% in the lesions with ≥ 5). No significant difference in the percentage of clonal cases was found between T1/T2 and T3/T4 lesions with a histopathological score ≥ 5. The multivariate logistic regression showed that the density and extent of the cell infiltrate, the degree of epidermotropism and the presence of cytological atypia share an independent predictive value for clonality in T1/T2 samples, even if the highest odds ratios (3.6) were associated with the density of the cell infiltrate. The disease course of T1/T2 patients was analysed according to the PCR findings. All the PCR- negative patients showed a long- standing stable disease course; on the other hand, a disease progression occurred in 12/87 (13.8% ) positive patients. Conclusions: The multiplex PCR/HD analysis is associated with a high diagnostic accuracy (92.7% ) in CTCL patients. The finding of a clonal T- cell rearrangement is more closely associated with the histological pattern (in particular with the density and extent of the cell infiltrate) rather than with the MF cutaneous T- score or immunophenotype.
