Primary cutaneous B-cell lymphoma: classification and treatment

Abstract

There has been confusion and debate regarding the definition, terminology, and optimal treatment of the different types of primary cutaneous B-cell lymphomas. This review presents the new World Health Organization-European Organization for the Research and Treatment of Cancer classification for cutaneous lymphomas; describes clinicopathologic, immunophenotypic, and genetic features of the different types of cutaneous B-cell lymphomas in this classification; and discusses current views on treatment of these lymphomas. The three main types of cutaneous B-cell lymphomas in this new classification are primary cutaneous marginal zone B-cell lymphoma, primary cutaneous follicle center lymphoma, and primary cutaneous large B-cell lymphoma (leg type). Primary cutaneous marginal zone B-cell and primary cutaneous follicle center lymphoma are indolent types with an excellent prognosis that should be treated primarily with nonaggressive therapies. Primary cutaneous large B-cell lymphoma (leg type) is an aggressive lymphoma that should be treated primarily with aggressive chemotherapy. The World Health Organization-European Organization for the Research and Treatment of Cancer classification will contribute to uniform diagnosis, management, and treatment of patients with cutaneous B-cell lymphoma and will prevent patients with indolent types of the disease from being treated inappropriately with systemic chemotherapy.