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Background: Mycosis fungoides (MF) is the most common types of cutaneous T cell lymphoma. It typically presents with erythematous patches and plaques in the early stage and tumors and extracutaneous involvement with possibly fatal outcomes in the late stage. To facilitate early and accurate diagnosis of MF, it is essential to be knowledgeable of classic and variants of this disease. However, there is limited published data in the Chinese population. Objective: To characterize our patient group and to provide additional insight into these malignancies. Methods: Patients diagnosed with mycosis fungoides and its variants from October 2012 to January 2018 were retrospectively analyzed. Disease-specific survival (DSS) rate and curve according to early and advanced stages were also calculated. Results: The mean age at diagnosis of ninety-three MF patients was 38.9±1.73 years (range: 6∼77). Forty-five males (48.4%) and 48 females (51.6 %) were included in this study. The DSS rate of early- stage MF was 98.6%, while that of advanced stage MF was 88.9%. There was a significant difference in DSS rate between early stage and advanced stage MF (p=0.042, logrank test). The median age of 10 patients with hypopigmented MF (hMF) was 10.5 years (range: 6∼28). The age of hMF was younger than that of classical MF patients (p ＜0.05). Conclusion: Early-stage MF has a better prognosis than advanced stage and hMF affects younger people than classic MF among Chinese. This study provides an insight into mycosis fungoides and its variants in a Chinese population. (Ann Dermatol 32(1) 14∼20, 2020)

// Chenchen Xu 1, * , Dan Zhou 2, * , Yixin Luo 1 , Shuai Guo 2 , Tao Wang 1 , Jie Liu 1 , Yuehua Liu 1 and Zhili Li 2 1 Department of Dermatology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China 2 Department of Biophysics and Structural Biology, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100005, China * These authors have contributed equally to this work and should be considered co-first authors Correspondence to: Jie Liu, email: Liujie04672@pumch.cn Yuehua Liu, email: yuehualiu@263.net Zhili Li, email: lizhili@ibms.pumc.edu.cn Keywords: cutaneous T cell lymphoma, mycosis fungoides, mass spectrometry imaging, matrix-assisted laser desorption/ionization-Fourier transform ion cyclotron resonance mass spectrometry, lipidomics Received: December 23, 2016 Accepted: May 04, 2017 Published: May 26, 2017 ABSTRACT Mycosis fungoides (MF) is the most common type of cutaneous T cell lymphoma. In this study, we used matrix-assisted laser desorption/ionization-Fourier transform ion cyclotron resonance mass spectrometry (MALDI-FTICR–MS) to perform lipidomic profiling of 5 MF tissue samples and 44 serum samples (22 from MF patients and 22 from control subjects). Multivariate statistical analysis of the mass spectral data showed that MF tissues had altered levels of seven lipids and MF sera had altered levels of twelve. Among these, six phosphotidylcholines, PC (34:2), PC (34:1), PC (36:3), PC (36:2), PC (32:0), and PC (38:4) and one sphingomyelin, SM (16:0) were altered in both MF tissues and sera. PC (34:2), PC (34:1), PC (36:3), and PC (36:2) levels were increased in both tissues and sera from MF patients, whereas SM (16:0), PC (32:0), and PC (38:4) levels were increased in MF sera but were decreased in MF tissues. We have thus identified multiple lipids that are altered in MF tissues and sera. This suggests serological and tissue lipidomic profiling could be an effective approach to screening for diagnostic biomarkers of MF.

Objective To determine the efficacy of palliative radiotherapy in treating tumor-stage cutaneous T-cell lymphoma/mycosis fungoides (MF). Methods From January 2008 to January 2013, a total of 11 patients with tumor-stage MF were treated with local radiation therapy in Peking Union Medical College Hospital. The median age of these patients was 53.36±14.45 years. Female-male ratio was 1:1.2. The average course of disease was 10.82±3.37 years. All the patients were treated with local electronic beam irradiation with a total median dosage of 48.55±9.51 (40-74) Gy in an average of 24.55±5.57 (20-40) fractions, 5 fractions per week. Results The median follow-up time was 55.27±29.3 (13-103) months. No severe acute or chronic side effects of irradiation were observed. Complete clinical response (CR) rate of the radiated sites was 54.5% (6/11), partial response (PR) rate was 36.4% (4/11), and the overall response rate (CR+PR) was 90.9%. One patient showed no response. Conclusion Local radiotherapy with psolaren plus ultraviolet A and/or interferon maintaining treatment is an effective palliative therapy in the treatment of tumor-stage MF patients.

Background Chidamide (CS055) is a new oral benzamide type of histone deacetylase (HDAC) inhibitor with subtype selective activity against HDAC1, 2, 3 and 10, and it has been approved for relapsed or refractory peripheral T-cell lymphoma (PTCL) in China. This phase II study (ChiCTR-TNC-00000806) was to evaluate the efficacy and safety of chidamide in different dosing regimens in patients with relapsed or refractory cutaneous T-cell Lymphoma (CTCL). Methods Fifty-two patients, all were mycosis fungoides or Sezary syndrome, were enrolled in this study. In the first stage of the study, 26 patients were randomly assigned to receive chidamide 30 mg twice per week for 2 weeks out of a 3-week-cycle (13 patients), or 4 weeks out of a 6-week-cycle (13 patients). Another 26 patients were enrolled in the second stage, and were treated by chidamide of 30 mg twice per week without drug-free holiday. The primary endpoint was objective response rate (ORR). Responses were evaluated based on the overall skin lesions, lymph nodes, blood cells and pruritis. Results The ORRs were 33% (4/12 PRs) for the 3-week-cycle arm, 23% (3/13 PRs) for the 6-week-cycle arm, and 36% (1/25 CR + 8/25 PRs) for the successive dosing arm, respectively. Median duration of response (DOR) was 50, 92, and 169 days for the indicated 3 arms, respectively. Twelve (92%), 11 (85%) and 20 (77%) patients experienced adverse event (AE) in the three arms. Most AEs were in Grade 1 or 2. Thrombocytopenia, leucopenia, fatigue, nausea and diarrhea were most frequently seen. Two serious adverse events (SAE) were reported in the study. One patient in the 3-week-cycle arm was hospitalized for fever and lung infection, and the other in the successive dosing arm was hospitalized for hyperglycemia. Conclusions Chidamide was active and tolerable to relapsed or refractory CTCL. The ORR of chidamide was comparable with the marketed drugs for CTCL. The major toxicities of chidamide were hematologic and gastrointestinal reactions which were controllable. Based on the overall profiles of the three different dosing regimens, 30 mg twice per week successively was clinically recommended. Table 1. Efficacy 3-week-cycle arm(N=12) * 6-week-cycle arm (N=13) Successive dosing arm(N=25) * CR 0 0 1 PR 4 3 8 ORR (%) 4 (33) 3 (23) 9 (36) Median DOR (days) 50 92 169 Median PFS (days) 84 81 88 *patients with ineligible entrance of the study were excluded for the efficacy analysis Table 2. Safety 3-week-cycle arm (N=13) 6-week-cycle arm (N=13) Successive dosing arm (N=26) Patients with AE (%) 12 (92) 11(85) 20 (77) Patients with AE ≥grade 3 (%) 3 (23) 5 (38) 4 (15) Patients with SAE (%) 1 (8) 0 1 (4) AEs in ≥10% of patients (%) Thrombocytopenia 5 (39) 3 (23) 9 (35) Leucopenia 7 (54) 4 (31) 4 (15) Fatigue 3 (23) 3 (23) 3 (12) Nausea 3 (23) 1 (8) 3 (12) Diarrhea 1(8) 1 (8) 3 (12) Fever 0 2 (15) 2 (8) Anemia 1 (8) 2 (15) 1 (4) Disclosures No relevant conflicts of interest to declare.