Treatment of Early Folliculotropic Mycosis Fungoides with Special Focus on Psoralen plus Ultraviolet A

Abstract

Data on the treatment of early folliculotropic mycosis fungoides, a recently defined clinicopathological subgroup of folliculotropic mycosis fungoides with an indolent course, is limited. Treatment outcomes were studied in a retrospective cohort of 47 adults with early folliculotropic mycosis fungoides, with a focus on psoralen plus ultraviolet A (PUVA) monotherapy, including dosimetric data, and the findings were compared with data for PUVA in 18 adults with early-classic mycosis fungoides. PUVA was given to 27 patients with early folliculotropic mycosis fungoides: 70% achieved complete response and 26% partial response. Significantly more treatments were needed to achieve complete response in stage IB compared with stage IA. There was no significant difference in the complete response rate from classic plaque-stage disease, although the early folliculotropic mycosis fungoides group required more treatments to achieve complete response, and a higher cumulative dose of UVA. Thus, PUVA is an effective treatment for early folliculotropic mycosis fungoides. Its complete response rate might be equal to early-classic mycosis fungoides; however, a longer induction phase is needed to achieve complete response.

Treatment of early‐stage mycosis fungoides: results from the PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study*

“…It is conceivable that some of these cases of FMF had infiltrated rather than thin plaques, thus representing advanced-stage FMF. [31][32][33] We have shown that the majority of patients with earlystage MF have persistent skin lesions after their first-line treatment (26% CR), which could potentially impact on their QoL. Our results indicate that half of the patients with early-stage disease (52%) had moderate-to-severe QoL reduction, in agreement with our recent report from the PROCLIPI database.…”

“…and the Dutch Cutaneous Lymphoma Group32 showed that FMF presents with two distinct patterns: early (follicle‐based patch or flat plaques) and advanced (follicle‐based infiltrated plaques and/or tumours). The good prognosis of early‐stage FMF implies that these patients should benefit from SDT 28,29,33. In our study, 18% of patients with early‐stage MF had FMF, and these patients were more likely to receive systemic first‐line therapies.…”

“…In our study, 18% of patients with early‐stage MF had FMF, and these patients were more likely to receive systemic first‐line therapies. It is conceivable that some of these cases of FMF had infiltrated rather than thin plaques, thus representing advanced‐stage FMF 31,32,33…”

Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy

“…In general, skin-directed therapies are used to treat early stages of MF and FMF as a first-line treatment, while systemic therapies, usually combined with skin-directed treatment, are more commonly used to treat more advanced stages. The treatment for FMF can be similar to that for classic MF, but the time to response and treatment duration in early FMF is more prolonged [104]. With regard to the other MF variants, PR and GSS, treatment with radiotherapy or surgical excision has been recommended [12,105,106] Corticosteroids impair not only lymphocyte binding to the endothelium, but also intercellular adhesion.…”

Stage-dependent Increase in Expression of miR-155 and Ki-67 and Number of Tumour-associated Inflammatory Cells in Folliculotropic Mycosis Fungoides

“…Early FMF is characterized by more superficial clinical features and less heavy perifollicular infiltrates than advanced/tumour-stage FMF. While the course of early FMF is indolent, similar to that of classic early-stage MF, and patients may respond to skin-targeted therapy, tumour-stage FMF has a more aggressive course similar to classic tumour-stage MF, and requires more aggressive treatments (7)(8)(9)(10).…”