Background

Mycosis fungoides (MF) and Sézary syndrome (SS) are forms of primary cutaneous T-cell lymphomas. The clinical aspects and symptoms vary greatly depending on the stage of disease. These are rare diseases which remain incurable, but there is a wide variety of treatment options. Few studies have been performed on this topic in Sweden and there is an emerging need for descriptive studies and research in this field.

Aim

The aim of this study is to describe and analyze diagnostic criteria for patients with MF/SS and to describe treatment modalities and evaluate treatment response of this patient-group.

Method

This is a descriptive retrospective study based on the data collected in the Primary Cutaneous Lymphoma quality register at the department of Dermatology and Venerology at Sahlgrenska University Hospital between 1997-2021.

Results

Eighty-two patients, median age 55.9 years (range 8.6– 91.7) at diagnosis, with MF (n=70) or SS (n=12) were included. Male to female ratio was 1.7:1. From the onset of skin symptoms until initial diagnosis took on average 2.7 years (median, range 0.1–42.4 years). The subtypes of MF were folliculotropic (n=9), syringotropic (n=4), hypopigmented (n=1) and hyperpigmented (n=1).
The majority of the patients (n=63) were diagnosed at early-stage disease (IA-IIA) while 18 patients were diagnosed with advanced stage disease (IIB-IVB). Overall disease progression was seen in 10% (n=8) of the patients. Eight (10 %) patients were deceased out of which three (3.8 %) deaths were associated with MF/ SS related causes.
The most common co-morbidities in our study population were overweight 31%, hypertension (29%), obesity (18%), hyperlipidaemia (16%), diabetes mellitus (15%), and dermatitis (11%). The most common malignancies were basal cell carcinoma (16%), haematological malignancies (7%), malignant melanoma (6%) and prostate cancer (6%).
Topical corticosteroids (96.3 %), UVB (57.3 %) and PUVA (47,6%) were common choices of treatment in all patients, along with acitretin in those with advanced stage disease. No significant difference in overall response was detected between patients with early-stage disease and advanced stage disease for any treatment.

Conclusion

This study contributes to knowledge on epidemiological and clinical features in addition to diagnostic findings in patients with MF and SS diagnosed and/or followed up for a long period of time in Western Sweden. We observed increased risk of cardiovascular disease and higher risk of developing other malignancies. This study suggests that treatment for these patients have been adequately adjusted for stage and highlights the importance of topical corticosteroids as an integrated part of the treatment. UVB and PUVA are beneficial treatments for patients with early-stage disease, but could also be valuable for patients with advanced disease along with acitretin. Hopefully this study will inspire to and be integrated in future research ahead.