Mycosis fungoides and Sézary syndrome

Hwang, Samuel T; Janik, John E.; Jaffe, Elaine S.; Wilson, Wyndham H.

doi:10.1016/s0140-6736(08)60420-1

Cited by 243 publications

(214 citation statements)

References 138 publications

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“…However, how these changes affect the T cell activities are still not clear. 3 Genetic disorder of NAV3 expression (the tumor suppressor) was found in 50-85% patient. Mutations of p53, p15, p16, junB and PTEN gen were observed in advance cases.…”

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confidence: 99%

“…2,4 There are trias in clinical symptom of SS which consist of erythroderma, lymphadenopathy and the typical Sezary cell. 3,5 Itchy skin lesions and thick squamae especially over the palmar and plantar areas, often occurred along with alopecia. Histopathologically, the SS shows non-specific inflammatory dermatosis, but in immuno-histochemistry staining some atypical CD4 T-cells are found with the increase ratio of CD4 / CD8 recorded at more than 10.…”

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confidence: 99%

“…Histopathologically, the SS shows non-specific inflammatory dermatosis, but in immuno-histochemistry staining some atypical CD4 T-cells are found with the increase ratio of CD4 / CD8 recorded at more than 10. 3,6,7 The prognosis of SS is not suitable with low remission and slow response to the few available treatment modalities. 3,8,9 case A 60 years old man was hospitalized in the Dermatology ward of Dr. Soetomo Hospital, complaining about itchy and thick patches all over the body in the last 3 days before admission.…”

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confidence: 99%

“…3,6,7 The prognosis of SS is not suitable with low remission and slow response to the few available treatment modalities. 3,8,9 case A 60 years old man was hospitalized in the Dermatology ward of Dr. Soetomo Hospital, complaining about itchy and thick patches all over the body in the last 3 days before admission. The erythematous plaques started in the back for a year previously and spread to the other parts of the body.…”

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confidence: 99%

“…The Ultra Violet treatment has been used to control the plaques; while the systemic treatment is used when topical therapy shows no progress. 3,8,9 Methotrexate (MTX) and other antimitotic drugs are the other available choices to manage SS. 14 In this case, MTX was only applied for one cycle and had to be discontinued due to the clinically worsening condition of the patient and the disturbance of his liver function.…”

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confidence: 99%

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Sezary Syndrome Mimicking Generalized Psoriasis Vulgaris

Lynoora

Rahmadewi

2017

IJTID

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Sezary Syndrome Mimicking Generalized Psoriasis Vulgaris

Lynoora

Rahmadewi

2017

IJTID

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Evaluation of Low-Dose, Low-Frequency Oral Psoralen–UV-A Treatment With or Without Maintenance on Early-Stage Mycosis Fungoides

et al. 2019

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Psoralen-UV-A (PUVA) photochemotherapy is standard first-line treatment for skin-limited, early-stage mycosis fungoides capable of producing high initial complete response (CR) rates. However, much remains unknown about PUVA's therapeutic mechanisms, optimal duration and frequency of treatment, dose escalation, or use as maintenance therapy. OBJECTIVES To evaluate low-dose, low-frequency PUVA, and whether maintenance treatment extends disease-free remission in patients with mycosis fungoides. DESIGN, SETTING, AND PARTICIPANTS This prospective randomized clinical trial with defined PUVA dosing regimen was carried out in 5 centers (Graz, Vienna, Hietzing, Innsbruck, and Salzburg) across Austria. Patients with stage IA to IIA mycosis fungoides (n = 27) were enrolled in the study beginning March 13, 2013, with the last patient enrolled March 21, 2016. These patients were treated with oral 8-methoxypsoralen followed by UV-A exposure 2 times per week for 12 to 24 weeks until CR. Patients with CR were randomized to PUVA maintenance for 9 months (14 total exposures) or no maintenance. The study was conducted from April 27, 2012, to July 27, 2018. Data analysis of the primary end point was of the intention-to-treat population, and the secondary end point analysis was of the evaluable population. MAIN OUTCOMES AND MEASURES Efficacy of the PUVA regimen was determined by the rate of CR as defined by a modified severity-weighted assessment tool (mSWAT) score reduction to 0. Levels of proinflammatory molecules in serum and histologic features and percentage of clonal T cells in skin were assessed to search for biomarkers of clinical response. RESULTS In 27 patients with mycosis fungoides, 19 (70%) were male with mean (range) age 61 (30-80) years. At baseline, patients with CR had a mean (range) mSWAT score of 18.6 (1-66) compared with 16.8 (3-46) in patients with partial response. The 12-to 24-week PUVA induction regimen reduced the mSWAT score in all patients and led to CR in 19 (70%) of 27 patients and a low mean cumulative UV-A dose of 78.5 J/cm 2. The subsequent standardized 9-month PUVA maintenance phase prolonged median (range) disease-free remission from 4 (1-20) months to 15 (1-54) months (P = .02). High density of histologic infiltrate and high percentage of clonal TCR sequences in skin biopsy specimens at baseline were inversely associated with therapeutic response. No severe adverse effects were seen during the PUVA induction or maintenance phase. CONCLUSIONS AND RELEVANCE This proof-of-concept study identifies potential biomarkers for therapeutic response to PUVA in mycosis fungoides; it also demonstrates that low-dose, low-frequency PUVA appears to be highly effective, and maintenance treatment may extend disease-free remission. TRIAL REGISTRATION ClinicalTrials.gov identifier: NCT01686594

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