罕见病研究 (Apr 2023)

Expert Guidelines for the Diagnosis, Treatment, and Management of Mycosis Fungoides in China

  • Rare Skin Disease Committee of China Alliance for Rare Diseases,
  • National Clinical Research Center for Dermatologic and Immunologic Diseases,
  • Dermatology Branch of China International Exchange and Promotion Association for Medical and Health Care

DOI
https://doi.org/10.12376/j.issn.2097-0501.2023.02.008
Journal volume & issue
Vol. 2, no. 2
pp. 191 – 209

Abstract

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Mycosis fungoides (MF) is a cutaneous lymphoma originating from memory helper T cells. The lesion caused by classical type of MF is characterized by the progression from patches at early stages, advancing to more infiltrated plaques and eventually to tumors or erythroderma. Lymph nodes and visceral organs may be involved. The clinical course can last for decades. Early diagnosis of MF is difficult, usually requiring regular follow-up, repeated skin biopsy, and comprehensive analysis of the clinical manifestations, and involving histopathology, immunophenotype, and molecular biology. The treatment of MF should be determined on the stage of the disease. Patients with early-stage MF should be treated with skin-directed therapy, such as topical drugs, phototherapy, and radiotherapy. Advanced-stage MF or recurrent and refractory early-stage MF needs systemic treatments which can be combined with skin-directed treatment to alleviate symptoms. Multidisciplinary treatment (MDT) model is important for the management of patients with MF. The MDT team should conduct an overall evaluation of the patients when formulating the treatment plan, fully weighing the possible benefits, patient tolerance and side effects of treatment, so as to delay the progress of the disease and improve the quality of life of patients. With reference to the latest international and national research data, combined with the true state of China and expert experience, we developed the guidelines to standardize the process of diagnosis, treatment, and management of MF in China.

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