Clinical characteristics, risk factors and long-term outcome of 114 patients with folliculotropic mycosis fungoides

Abstract

Folliculotropic mycosis fungoides (FMF) is a distinct variant of mycosis fungoides (MF) where atypical T-cells invade the hair follicles. The objective was to assess the clinical features, risk factors for progression, long-term outcome and response to treatment modalities in a large cohort of FMF patients. We, therefore, conducted a single-center retrospective study, reviewing 114 patients with FMF seen from 1987 to 2015 at the cutaneous T-cell lymphoma clinic of the MD Anderson Cancer Center. The mean age at diagnosis of FMF was 57.1 ± 13.5 years. The male to female ratio was 1.2:1. MF stage IA (n = 50, 43.9%) was the most common diagnosed stage, followed equally by stages IB and IIB (n = 23, 20.2%, respectively). Eighty-six patients (75.4%) suffered from pruritus. Concomitant hair loss was observed in 37 (32.5%) subjects. The median number of different treatment modalities used per patient was 4 (range 1-12). Low-dose radiation was beneficial in clearing therapy refractory lesions. The overall survival was influenced by several factors, including advanced age (>65), late stages and the presence of large cell transformation (LCT). Thirty-three of 113 patients (29.2%) progressed to more advanced stages and 26 (23.0%) died, with the cause of death being MF in 11 (9.7%) subjects. In conclusion, patients with FMF have a worse overall 10-year survival rate compared with other MF variants. Several factors, including stage, advanced age and the presence of LCT impact survival. Multiple different treatment approaches may be needed to achieve a good clinical response and to prevent disease progression.

Folliculotropism Does Not Affect Overall Survival in Mycosis Fungoides: Results from a Single-Center Cohort and Meta-Analysis

“…Predictors of poor survival were advanced age, male sex, and stage >IB. Our study supports the previous literature, which suggests that advanced age and male sex are associated with poor survival [2,5,[13][14][15][16] . This is in keeping with the cutaneous lymphoma international prognostic index (CLIPi) for MF and SS, which lists male gender and age >60 as significant adverse prognostic factors [17] .…”

Folliculotropic mycosis fungoides

“…Common combinations include topical corticosteroids and narrowband UVB (311 nm) or PUVA therapy. If the response is inadequate, the addition of oral bexarotene has been shown to be beneficial . Other systemic options are interferon alpha or methotrexate (MTX).…”

“…Dabei werden häufi g topische Steroide mit einer UVB-311-oder einer PUVA-Therapie kombiniert. Bei unzureichendem Ansprechen hat sich die Zugabe von oralem Bexaroten bewährt[ 37,38 ] . Andere Systemtherapeutika wie Interferon-alpha oder Methotrexat (MTX) können ebenfalls zur Anwendung kommen.…”

Die follikulotrope Mycosis fungoides

“…Dabei werden häufig topische Steroide mit einer UVB‐311‐ oder einer PUVA‐Therapie kombiniert. Bei unzureichendem Ansprechen hat sich die Zugabe von oralem Bexaroten bewährt . Andere Systemtherapeutika wie Interferon‐alpha oder Methotrexat (MTX) können ebenfalls zur Anwendung kommen.…”