Folliculotropic mycosis fungoides: Clinical characteristics, treatments, and long-term outcomes of 53 patients in a tertiary hospital

Kalay Yildizhan İ., ŞANLI H., Akay B. N., Uzundere C., Okcu Heper A.

Dermatologic Therapy, cilt.33, sa.4, 2020 (SCI-Expanded) identifier identifier identifier

  • Yayın Türü: Makale / Tam Makale
  • Cilt numarası: 33 Sayı: 4
  • Basım Tarihi: 2020
  • Doi Numarası: 10.1111/dth.13585
  • Dergi Adı: Dermatologic Therapy
  • Derginin Tarandığı İndeksler: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Anahtar Kelimeler: clinical features, cutaneous T-cell lymphoma, folliculotropic mycosis fungoides, prognosis, therapy, SINGLE-CENTER, PROGNOSTIC-FACTORS, INTERNATIONAL-SOCIETY, FEATURES, CLASSIFICATION, ORGANIZATION, SURVIVAL, SERIES
  • Ankara Üniversitesi Adresli: Evet

Özet

Folliculotropic mycosis fungoides (FMF) is characterized by a broad clinical spectrum and worse prognosis compared to classical MF. This study aimed to evaluate the clinical characteristics, treatment modalities and long-term outcome and risk factors for progression and survival of FMF patients. We conducted a single-center retrospective study and reviewed 53 patients diagnosed with FMF between 1990 to 2019 in a referral center at Ankara University, Turkey. Regarding to stage at diagnosis, 24 patients (45.3%) had advanced-stage disease (≥IIB). Follicular papules was observed in 66% and alopecia in 49.1% of the cases. Forty-three patients (81.1%) suffered from pruritus. The majority of the patients (92.4%) had at least one systemic therapy. Complete remission was achieved in 24.5% of the patients. The median time of overall survival (OS) was 50 months (range 9-324 months) and 5-year and 10-year OS was 83% and 69%, respectively. Twenty-eight (52.3%) patients progressed to more advanced stages and seven (13.2%) patients died due to MF during the follow-up period. FMF is associated with a progressive course and in most patients, skin-directed therapies were found to be inefficient to control the disease and multiple systemic therapeutic agents were required to control the disease.