Quality of life in patients with Mycosis Fungoides and Sézary Syndrome: a systematic review of the literature

Abstract

Cutaneous T‐cell Lymphoma’s (CTCL) are a rare, heterogeneous group of T‐cell lymphomas that primarily manifest in the skin. Mycosis fungoides (MF) and Sézary syndrome (SS) are considered the classic types of CTCL. The diverse manifestation of CTCL results in a wide range of symptoms with a possible mild to severe impact on Quality of Life (QoL) depending on the disease stage. Previous studies on QoL in CTCL patients report diverse patient populations and use many different QoL instruments. In the current literature, a clear overview on the influence of the different stages of disease (early MF, late‐stage MF/SS or total group) on the QoL is lacking. Therefore, a systematic search of the literature was conducted using the PubMed, Embase, PsycINFO and Web of Science databases. Studies were included if they described QoL in patients with MF and SS retrieved by standardized instruments or qualitative interviews. In total, 24 studies were included using 18 different questionnaires to report on dermatology‐specific, cancer‐specific and generic QoL. The effect on QoL was found to be greater in patients with late‐stage disease as compared to early stage disease, with significant impairments on functional, emotional and physical domains. Nonetheless, even in patients with limited disease, QoL was mildly to moderately affected. Overall, pruritus was the most frequent reported and most bothersome symptom. Significant influence of the disease on daily life activities were found, not only in patients but also on caregivers and family. This broad, structured overview on QoL in MF and SS patients underlines the influence of disease stage on QoL, and therefore, recommends future studies to distinguish between disease stages when reporting results. Furthermore, this overview can inform clinicians in clinical practice by creating awareness of QoL deficits according to disease stage.

Mast cells and tryptase are linked to itch and disease severity in mycosis fungoides: Results of a pilot study

“…Taken together, our results indicate that itch is an important clinical marker in MF which can be easily assessed using simple questionnaires (7,32) and could be even explored as a tool for disease severity monitoring. Furthermore, our report suggests that MC and their mediator tryptase play a role in itch in MF.…”

“…Itch can be a bothersome symptom and affects more than half of the patients with MF. Itch, in MF, is one of the main drivers of quality of life impairment in these patients (6)(7)(8). The exact mechanisms of itch induction in MF lesions are not understood.…”

The Role of Tumor Microenvironment in the Pathogenesis of Sézary Syndrome

“…The most important symptom in SS patients is pruritus. It is observed in virtually all patients with SS, and its intensity is directly correlated with a reduction in the quality of life [3,124]. Nerve growth factor (NGF) is produced by keratinocytes, stimulates nerve fibers growth, and is associated with the severity of pruritus.…”

Itch in patients with cutaneous T-cell lymphoma as a quality of life indicator

“…A recent systematic review shows that CTCL has a significant effect on quality of life (QoL), with a more profound effect in patients with more advanced disease. 4 However, few studies account for the different stages of CTCL on the different domains of QoL. 4 Awareness of the specific affected domains in patients with CTCL could help to improve QoL.…”

“… 4 However, few studies account for the different stages of CTCL on the different domains of QoL. 4 Awareness of the specific affected domains in patients with CTCL could help to improve QoL. Furthermore, the QoL in patients with FMF is not yet investigated.…”