Phototherapy for mycosis fungoides. 2015

Sunil Dogra, and Rahul Mahajan
Department of Dermatology, Venereology and Leprology, Post-Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

BACKGROUND Both phototherapy and photochemotherapy have been used in all stages of mycosis fungoides since they improve the symptoms and have a favourable adverse effect profile. METHODS We performed an extensive search of published literature using keywords like "phototherapy", "photochemotherapy", "NBUVB", "PUVA", "UVA1", "mycosis fungoides", and "Sezary syndrome", and included systematic reviews, meta-analysis, national guidelines, randomized controlled trials (RCTs), prospective open label studies, and retrospective case series. These were then arranged according to their levels of evidence. RESULTS Five hundred and forty three studies were evaluated, of which 107 fulfilled the criteria for inclusion in the guidelines. CONCLUSIONS Photochemotherapy in the form of psoralens with ultraviolet A (PUVA) is a safe, effective, and well tolerated first line therapy for the management of early stage mycosis fungoides (MF), that is, stage IA, IB, and IIA (Level of evidence 1+, Grade of recommendation B). The evidence for phototherapy in the form of narrow-band UVB (NB-UVB) is less robust (Level of evidence 2++, Grade of recommendation B) but may be considered at least as effective as PUVA in the treatment of early-stage MF as an initial therapy. In patients with patches and thin plaques, NB-UVB should be preferentially used. PUVA may be reserved for patients with thick plaques and those who relapse after initial NB-UVB therapy. For inducing remission, three treatment sessions per week of PUVA phototherapy or three sessions per week of NB-UVB phototherapy may be advised till the patient achieves complete remission. In cases of relapse, patients may be started again on PUVA monotherapy or PUVA may be combined with adjuvants like methotrexate and interferon (Level of evidence 2+, Grade of recommendation B). Patients with early-stage MF show good response to combination treatments like PUVA with methotrexate, bexarotene or interferon-α-2b. However, whether these combinations hold a significant advantage over monotherapy is inconclusive. For late stage MF, the above-mentioned combination therapy may be used as first-line treatment (Level of evidence 3, Grade of recommendation C). Currently, there is no consensus regarding maintenance therapy with phototherapy once remission is achieved. Maintenance therapy should not be employed for PUVA routinely and may be reserved for patients who experience an early relapse after an initial course of phototherapy (Level of evidence 2+, Grade of recommendation B). Bath-water PUVA may be tried as an alternative to oral PUVA in case the latter cannot be administered as the former may show similar efficacy (Level of evidence 2-, Grade of recommendation C). In pediatric MF and in hypopigmented MF, both NB-UVB and PUVA may be tried (Level of evidence 3, Grade of recommendation D).

UI MeSH Term Description Entries
D009182 Mycosis Fungoides A chronic, malignant T-cell lymphoma of the skin. In the late stages, the LYMPH NODES and viscera are affected.
D010789 Phototherapy Treatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths. Blue Light Therapy,Blue-light Therapy,Light Therapy,Photoradiation Therapy,Red Light Phototherapy,Therapy, Photoradiation,Blue Light Therapies,Blue-light Therapies,Light Phototherapies, Red,Light Phototherapy, Red,Light Therapies,Light Therapies, Blue,Light Therapy, Blue,Photoradiation Therapies,Phototherapies,Phototherapies, Red Light,Phototherapy, Red Light,Red Light Phototherapies,Therapies, Blue Light,Therapies, Blue-light,Therapies, Light,Therapies, Photoradiation,Therapy, Blue Light,Therapy, Blue-light,Therapy, Light
D011446 Prospective Studies Observation of a population for a sufficient number of persons over a sufficient number of years to generate incidence or mortality rates subsequent to the selection of the study group. Prospective Study,Studies, Prospective,Study, Prospective
D011701 PUVA Therapy Photochemotherapy using PSORALENS as the photosensitizing agent and ultraviolet light type A (UVA). Psoralen Ultraviolet A Therapy,Therapy, PUVA,PUVA Therapies,Therapies, PUVA
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012189 Retrospective Studies Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons. Retrospective Study,Studies, Retrospective,Study, Retrospective
D012878 Skin Neoplasms Tumors or cancer of the SKIN. Cancer of Skin,Skin Cancer,Cancer of the Skin,Neoplasms, Skin,Cancer, Skin,Cancers, Skin,Neoplasm, Skin,Skin Cancers,Skin Neoplasm
D014467 Ultraviolet Therapy The use of ultraviolet electromagnetic radiation in the treatment of disease, usually of the skin. This is the part of the sun's spectrum that causes sunburn and tanning. Ultraviolet A, used in PUVA, is closer to visible light and less damaging than Ultraviolet B, which is ionizing. Actinotherapy,Therapy, Ultraviolet,Actinotherapies,Therapies, Ultraviolet,Ultraviolet Therapies
D016032 Randomized Controlled Trials as Topic Works about clinical trials that involve at least one test treatment and one control treatment, concurrent enrollment and follow-up of the test- and control-treated groups, and in which the treatments to be administered are selected by a random process, such as the use of a random-numbers table. Clinical Trials, Randomized,Controlled Clinical Trials, Randomized,Trials, Randomized Clinical

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