No abstract

To evaluate the clinical and prognostic features in primary cutaneous CD8 + T-cell lymphomas, which are rare and considered to be aggressive cutaneous lymphoproliferative disorders.Design: Single-center retrospective study.Setting: Lymphoma clinic (referral center) of a university hospital.Patients: Three patients presented with CD8 + cutaneous lymphoma characterized by a patchlike pattern and hyperpigmentation.Results: Histological analysis revealed a CD3 + , CD8 + small-cell infiltrate showing a remarkable affinity to the dermoepidermal junction zone. Clonality for the T-cell receptor ␥ chain was detected by polymerase chain reaction followed by denaturing gradient gel electrophoresis. The clinical presentation lasted several years (6 and 9 years, respectively) before the correct diagnosis was made. Treatment with nontoxic approaches (UV-B and local steroids) was successful. Aggressive clinical behavior was not observed.Conclusions: Our 3 cases of junctional CD8 + cutaneous T-cell lymphomas were characterized by hyperpigmentation and nonaggressive clinical behavior. This type of lymphoma, which can be considered a CD8 + mycosis fungoides variant, must be distinguished from other types of cutaneous CD8 + lymphomas so that overtreatment can be avoided.

Cutaneous lymphomas include various types of clonal lymphoproliferative disorders. The adequate treatment approach depends on the exact diagnosis and should be non-aggressive in most cases. In early stages, local approaches such as UV or radiotherapy are preferred. In advanced stages, systemic drugs such as interferon-α or bexarotene can be administered. Experimental approaches for cutaneous lymphomas include vaccination and gene therapy.

No abstract

In cutaneous T cell lymphomas, tumor cells can be found in skin and in other compartments. A precise definition of extracutaneous spread including blood involvement is necessary for staging and treatment design. We investigated peripheral blood in 51 patients with various types of cutaneous T cell lymphomas by the analysis of blood smears for Sézary cells, the CD4 + /CD7- T helper cell frequency in the peripheral blood by fluorescence activated cell sorter analysis and by polymerase chain reaction for the T cell receptor gamma-chain followed by denaturing gradient gel electrophoresis. Eleven polymerase chain reaction products were sequenced. Thirty-five per cent of patients with stage Ia-IIb cutaneous T cell lymphomas presented a peripheral blood T cell clone. In patients with stage III-IVb cutaneous T cell lymphomas 75% were positive for clonality in the peripheral blood by polymerase chain reaction. Interestingly, three of 13 Sézary patients showed a TCR-gamma joining region pseudogene (JgammaP1/JgammaP2) usage. CD4 + /CD7- cell counts were significantly higher in patients with advanced cutaneous T cell lymphomas than in patients with early cutaneous T cell lymphomas. There was a correlation between increased percentage of circulating CD4 + /CD7- cells and detection of clonality by polymerase chain reaction (p = 0.001). There was no significant correlation between the polymerase chain reaction data and the percentage of Sézary cells on blood smears. A significant correlation between CD4 + /CD7- cells and Sézary cells was found, however. Stepwise logistic regression analysis showed that the CD4 + /CD7- cell count and clonal T cell detection in peripheral blood are independently correlated with stage. The combination of both parameters gives more information than each one separately. In conclusion, our data indicate that fluorescence activated cell sorter analysis of peripheral blood and polymerase chain reaction-based clonality assays can improve the accuracy of staging investigations in cutaneous T cell lymphomas patients.