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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.
Comment3 facts made this case interesting: (a) the leukemic differential blood count, which returned to normal; (b) the manifestation of a drug eruption as AGEP; and (c) the positive patch test. Patch testing with the suspected drug in cases of generalized pustular eruptions has been warned against (3), and exacerbations during testing described (4). Our patient produced a disseminated test reaction exceeding the patch test area. Pustular exanthemata have been reported by several authors as a manifestation of drug allergy (2). In 63 cases of acute generalized exanthematous pustulosis, antibiotics were . involved in 80% (5). The main difference between our case and others in the literature was the dramatic shift to the left in blood count, imitating leukemia. The more usual cutaneous side-effects of propicillin are urticaria Contact Dermatitis 1995: 33: 205 and maculopapular rashes, occurring in 1-5% of patients treated (I).
Summary
Background
The PROspective Cutaneous Lymphoma International Prognostic Index (PROCLIPI) study is a prospective analysis of an international database. Here we examine front‐line treatments and quality of life (QoL) in patients with newly diagnosed mycosis fungoides (MF).
Objectives
To identify (i) differences in first‐line approaches according to tumour‐nodes‐metastasis‐blood (TNMB) staging; (ii) parameters related to a first‐line systemic approach and (iii) response rates and QoL measures.
Methods
In total, 395 newly diagnosed patients with early‐stage MF (stage IA–IIA) were recruited from 41 centres in 17 countries between 1 January 2015 and 31 December 2018 following central clinicopathological review.
Results
The most common first‐line therapy was skin‐directed therapy (SDT) (322 cases, 81·5%), while a smaller percentage (44 cases, 11·1%) received systemic therapy. Expectant observation was used in 7·3%. In univariate analysis, the use of systemic therapy was significantly associated with higher clinical stage (IA, 6%; IB, 14%; IIA, 20%; IA–IB vs. IIA, P < 0·001), presence of plaques (T1a/T2a, 5%; T1b/T2b, 17%; P < 0·001), higher modified Severity Weighted Assessment Tool (> 10, 15%; ≤ 10, 7%; P = 0·01) and folliculotropic MF (FMF) (24% vs. 12%, P = 0·001). Multivariate analysis demonstrated significant associations with the presence of plaques (T1b/T2b vs. T1a/T2a, odds ratio 3·07) and FMF (odds ratio 2·83). The overall response rate (ORR) to first‐line SDT was 73%, while the ORR to first‐line systemic treatments was lower (57%) (P = 0·027). Health‐related QoL improved significantly both in patients with responsive disease and in those with stable disease.
Conclusions
Disease characteristics such as presence of plaques and FMF influence physician treatment choices, and SDT was superior to systemic therapy even in patients with such disease characteristics. Consequently, future treatment guidelines for early‐stage MF need to address these issues.
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