Primary cutaneous lymphomas encompass a spectrum of clinical and histologic variants, characterized by skin-homing lymphocytes. Awareness of their unique clinical behavior provides a stage-dependent treatment approach and minimizes unnecessary interventions. Systemic progression in CBCL is uncommon and 5-year survival rates are excellent, although relapses occur frequently. Treatment considerations for CBCL mostly rely on retrospective studies and are based on clinical presentation and morphologic evaluation. Histologic characterization of B-cell lymphomas in extranodular locations, such as the skin, can be problematic (i.e., small vs large cleaved cells), and does not appear to have the same predictive power compared to their nodal counterparts. Current clinical trials focus mainly on patients with MF/SS, as these patients represent the majority of cutaneous lymphomas. Skin-directed therapies are reserved for early stages.98 Immune-modifying therapies have emerged for the treatment of advanced and/or refractory stages. They have the potential to reconstitute immune function while augmenting host antitumor response.

Combined immunomodulatory regimens may result in sustained remission of disease. Our gold standard for advanced stages is the combined use of PUVA and INF-a at a dose of up to 9 MU thrice weekly or as tolerated. Systemic single-agent or multiagent chemotherapies should be reserved for more advanced and refractory disease. Clinical trials are ongoing to refine protocols for combination therapy to improve efficacy and minimize toxicity. Advances in molecular biology technology may allow selective targeting of both Band T-cell-mediated effects. Investigational agents, such as histone-deacetylase inhibitors, and the novel T-cell costimulatory agents, such as deoxynucleotide CpG7909 (cytosine-phosphorothiolated guanine-con-taining oligonucleotides) and lenalidomide, are being evaluated in phase I/II trials.

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