Characteristics associated with significantly worse quality of life in mycosis fungoides/Sézary syndrome from the Prospective Cutaneous Lymphoma International Prognostic Index ( PROCLIPI ) study

Abstract

Summary Background Mycosis fungoides (MF) and Sézary Syndrome (SS) are the most common cutaneous T‐cell lymphomas. MF/SS is accompanied by considerable morbidity from pain, itching and disfigurement. Aim To identify factors associated with poorer health‐related quality of life (HRQoL) in patients newly diagnosed with MF/SS. Methods Patients enrolled into Prospective Cutaneous Lymphoma International Prognostic Index (PROCLIPI; an international observational study in MF/SS) had their HRQoL assessed using the Skindex‐29 questionnaire. Skindex‐29 scores were analysed in relation to patient‐ and disease‐specific characteristics. Results The study population consisted of 237 patients [60·3% male; median age 60 years, (interquartile range 49–70)], of whom 179 had early MF and 58 had advanced MF/SS. In univariate analysis, HRQoL, as measured by Skindex‐29, was worse in women, SS, late‐stage MF, those with elevated lactate dehydrogenase, alopecia, high modified Severity Weighted Assessment Tool and confluent erythema. Linear regression models only identified female gender (β = 8·61; P = 0·003) and alopecia (β = 9·71, P = 0·02) as independent predictors of worse global HRQoL. Item‐level analysis showed that the severe impairment in symptoms [odds ratio (OR) 2·14, 95% confidence interval (CI) 1·19–3·89] and emotions (OR 1·88, 95% CI 1·09–3·27) subscale scores seen in women was caused by more burning/stinging, pruritus, irritation and greater feelings of depression, shame, embarrassment and annoyance with their diagnosis of MF/SS. Conclusions HRQoL is significantly more impaired in newly diagnosed women with MF/SS and in those with alopecia. As Skindex‐29 does not include existential questions on cancer, which may cause additional worry and distress, a comprehensive validated cutaneous T‐cell lymphoma‐specific questionnaire is urgently needed to more accurately assess disease‐specific HRQoL in these patients. What's already known about this topic? Cross‐sectional studies of mixed populations of known and newly diagnosed patients with mycosis fungoides (MF)/Sézary syndrome (SS) have shown significant impairment in health‐related quality of life (HRQoL). Previous studies on assessing gender‐specific differences in HRQoL in MF/SS are conflicting. More advanced‐stage disease and pruritus is associated with poorer HRQoL in patients with MF/SS. What does this study add? This is the first prospective study to investigate HRQoL in a homogenous group of newly diagnosed patients with MF/SS. In patients newly diagnosed with MF/SS, HRQoL is worse in women and in those with alopecia and confluent erythema. MF/SS diagnosis has a multidimensional impact on patient HRQoL, including a large burden of cutaneous symptoms, as well as a negative impact on emotional well‐being.

Blockade of programmed cell death protein 1 (PD-1) in Sézary syndrome reduces Th2 phenotype of non-tumoral T lymphocytes but may enhance tumor proliferation

“…Leukemic cutaneous T-cell lymphoma (L-CTCL), comprising Sézary Syndrome (SS) and mycosis fungoides (MF) with blood involvement, are common representatives of the heterogeneous group of primary cutaneous T-cell lymphomas, [1][2][3] which arise from malignant T cells homing to (SS) or residing in (MF) the skin. 4 In contrast to early stage skin limited MF, SS and patients with advanced MF with blood and/or organ involvement are characterized by poorer prognosis.…”

Management of primary cutaneous lymphoma patients during COVID‐19 pandemic: EORTC CLTF guidelines

“…19 Skin-directed therapies are still the most appropriate option for early-stage MF, and most patients in stage IA will have a normal life expectancy but morbidity from skin symptoms such as itch and pain, emotional disturbance from unsightly lesions and dealing with a non-curable disease reduced function that is from lesions on the hands can negatively impact on quality of life. 20 Low-risk patients especially those that do not receive systemic treatment are not more susceptible to infections than the general population. Patients in advanced stages with a defective immune system are more susceptible to opportunistic infections and probably to a more severe course of coronavirus infection.…”

Quality of life, Skindex‐29, mycosis fungoides/Sézary syndrome

“…Little is currently known about the health‐related quality of life (HRQoL) of patients with cutaneous T‐cell lymphoma (CTCL), characterized by chronic, pruritic skin lesions, a feature that may be distinctively influential. Although HRQoL plays a vital role in decision‐making and clinical treatment of CTCL, there are no specific, validated CTCL questionnaires …”

“…There have been few studies of the impact of mycosis fungoides (MF) and other CTCLs on patients’ HRQoL . No studies have included more than a relatively small series of patients, except in the current article, which has patients from all over the world.…”

“…This study is unique as it is the largest international study, with 25 centres, including four continents and patients from all over the world. This study evaluates HRQoL in patients newly diagnosed with MF and Sézary syndrome (SS), prospectively, using the Skindex‐29 questionnaire …”