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.
Leiden University Medical Center, Department of Dermatology, Leiden, The Netherlands
Correspondence to Rein Willemze, MD, Leiden University Medical Center, Department of Dermatology, B1-Q-93, PO Box 9600, 2300 RC Leiden, The Netherlands Tel: +31 71 526 2421; fax: +31 71 524 8106; e-mail: email@example.com