Cutaneous T-cell lymphoma

Abstract

Cutaneous T-cell lymphomas are a group of extranodal non-Hodgkin's lymphomas that present initially in the skin. The incidence of this condition is approximately 0.52-0.64/100,000 person-years. Cutaneous T-cell lymphomas usually present as indolent patches and plaques, but may also present with more aggressive tumors or erythroderm. The survival is highly dependent on the stage of the disease. The diagnosis is made with clinical findings and histology. Staging of this disease is critical, and this may include history and physical examination, blood tests, peripheral blood flow cytometry, and Sézary cell preparations, and scans for internal involvement. Cell trafficking of malignant T cells into the skin and the epidermis is critical in the pathophysiology of this disease, and involves chemokines and chemokine receptors. The immunology of this condition suggests that T-helper-2 (Th2) lymphocyte cytokine patterns are associated with the malignant cells, and there is a progressive loss of cellular immunity with advancing disease. There are two major categories of treatments for this condition: skin-directed therapies or systemic therapies. Combination therapies are effective and have immunomodulatory effects. Bone marrow transplantation is an emerging therapy for patients with advanced disease that is refractory to skin and systemic therapy. © 2008 Springer London.