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. 2021 Dec;35(12):2377-2387.
doi: 10.1111/jdv.17570. Epub 2021 Aug 14.

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

R Ottevanger  1 , S van Beugen  2 , A W M Evers  2 , R Willemze  1 , M H Vermeer  1 , K D Quint  1
Affiliations

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

R Ottevanger et al. J Eur Acad Dermatol Venereol. 2021 Dec.

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.

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Figure 1
Prisma Flowchart of systematic review of the literature.
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References

    1. Willemze R, Cerroni L, Kempf W et al. The 2018 update of the WHO‐EORTC classification for primary cutaneous lymphomas. Blood 2019; 133(16): 1703–1714. Blood. 2019; 134(13): 1112. - PMC - PubMed
    1. Willemze R, Jaffe ES, Burg G et al. WHO‐EORTC classification for cutaneous lymphomas. Blood 2005; 105(10): 3768–3785. - PubMed
    1. Kim YH, Hoppe RT. Mycosis fungoides and the Sezary syndrome. Semin Oncol 1999; 26(3): 276–289. - PubMed
    1. Ottevanger R, de Bruin DT, Willemze R et al. Incidence of Mycosis fungoides and Sezary syndrome in the Netherlands between 2000 and 2020. Br J Dermatol 2021; 185: 434–435. 10.1111/bjd.20048. - DOI - PMC - PubMed
    1. Demierre MF, Gan S, Jones J, Miller DR. Significant impact of cutaneous T‐cell lymphoma on patients' quality of life: results of a 2005 National Cutaneous Lymphoma Foundation Survey. Cancer 2006; 107(10): 2504–2511. - PubMed

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