Published online May 2, 2015. doi: 10.5314/wjd.v4.i2.69
Peer-review started: January 28, 2015
First decision: March 6, 2015
Revised: March 16, 2015
Accepted: April 8, 2015
Article in press: April 9, 2015
Published online: May 2, 2015
Mycosis fungoides is the most common form of cutaneous T-cell lymphoma (CTCL), and is characterized by a clonal expansion of malignant CD4+ T lymphocytes with skin-homing properties. Clinically and pathologically, mycosis fungoides can be categorized into patch, plaque and tumor stages. The clinical course of mycosis fungoides is usually chronic and indolent, but a proportion of patients may develop progressive disease with peripheral blood, lymph node and visceral organ involvement. Sézary syndrome is an aggressive leukemic form of CTCL characterized by a clonal population of malignant T cells in the peripheral blood. Various forms of skin-directed and systemic treatments are available for mycosis fungoides and Sézary syndrome. However, current treatments are generally not curative, and can only control the disease. Currently, the etiology and pathogenesis of mycosis fungoides and Sézary syndrome are not well defined. Proposed mechanisms include chronic antigenic stimulation by infectious agents, expression of specific adhesion molecules, altered cytokine production, mutations of oncogenes and tumor suppressor genes, and avoidance of apoptosis. In recent years, a number of chemokine receptors and their corresponding chemokine ligands have been found to contribute to the migration and survival of lymphoma cells in mycosis fungoides and Sézary syndrome, including CC chemokine receptor 4 (CCR4), CCR10, C-X-C chemokine receptor type 4 (CXCR4), CCR7, CCR3 and CXCR3. Since chemokines and chemokine receptors have been found to play important roles in the pathophysiology of mycosis fungoides and Sézary syndrome, they may be potentially useful targets for the development of new treatments for these diseases in the future.
Core tip: Mycosis fungoides and Sézary syndrome are characterized by a clonal expansion of malignant CD4+ T lymphocytes with skin-homing properties. Currently, treatment options for mycosis fungoides and Sézary syndrome are limited. The lack of effective targeted therapy results in part from the poor understanding regarding the pathophysiology of these diseases. Recently, a number of chemokines and chemokine receptors have been found to contribute to the pathogenesis of mycosis fungoides and Sézary syndrome, including the CC chemokine receptor 4 (CCR4)/chemokine (C-C motif) ligand 17 (CCL17), CCR10/CCL27, C-X-C chemokine receptor type 4/chemokine (C-X-C Motif) ligand 12 and CCR7/CCL21 axes. Therefore, these chemokines and chemokine receptors may be potentially useful targets for the treatment of these lymphomas in the future.