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Official Journal of the Italian Society of Dermatology and Sexually Transmitted Diseases
Indexed/Abstracted in: EMBASE, PubMed/MEDLINE, Science Citation Index Expanded (SciSearch), Scopus
Impact Factor 1,014
Online ISSN 1827-1820
Hoff N.-P., Bruch-Gerharz D..
Department of Dermatology, University Clinic of Duesseldorf, Germany
Cutaneous T-cell lymphoma represents a broad spectrum of disorders with various manifestations, clinical courses, and therapeutic considerations. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, the skin being affected by erythematous patches, plaques, or tumors. The related Sézary syndrome is a more aggressive form of cutaneous T-cell lymphoma with cutaneous and leukemic manifestations. Recent advances in the understanding of the molecular and biologic behavior of T cells in these disorders have facilitated the development of novel treatments, using biologic agents, phototherapeutic methods, and irradiation techniques. Nevertheless, there remains a continued need to develop new therapeutic options, particularly for patients with advanced-stage and treatment-refractory disease. At present, hematopoietic stem-cell transplantation has the best chance for expanding the therapeutic armamentarium of cutaneous T-cell lymphoma. In advanced-stage mycosis fungoides and the Sézary syndrome, stem cell transplantation may result in sustained remissions and improvement of progression-free survival. However, physicians have to be aware of severe clinical problems like graft-versus-host disease as well as increased risk of infection and secondary cancers. In this review, we focus on the role of hematopoietic stem cell transplantation for the therapy of mycosis fungoides and the Sézary syndrome.