* Authors on the Steering Committee contributed equally to the oversight of the study, including study design and maintaining the quality of study conduct. CONTRIBUTORS Owen O'Connor, Barbara Pro, Tim Illidge and Lorenz Trumper formed the ECHELON-2 steering committee and contributed equally to the oversight of the study, including study design and maintaining the quality of study conduct.
ScopeThis guideline is aimed at providing healthcare professionals with clear guidance on the management of patients with diffuse large B-cell lymphoma (DLBCL). Disease confined to specific extranodal sites, such as primary central nervous system lymphoma, testicular lymphoma, primary mediastinal large B-cell lymphoma, DLBCL of leg type, etc., is beyond the scope of this guideline. It is not the intention of this guideline to provide treatment recommendations for all situations and clinicians are advised to make management decisions taking into account individual patient circumstances.
MethodologyThe guideline group was selected to be representative of UK experts in the assessment and treatment of DLBCL. Recommendations are based on a systematic review of published English language literature up to January 2015. MEDLINE database was searched using the key words DLBCL, treatment, radiotherapy and transplant. References from relevant publications were also searched. Other published guidelines, including the US National Comprehensive Cancer Network (NCCN) and European Society for Medical Oncology guidelines, were also noted.The writing group produced a draft guideline. Review of the manuscript was performed by the British Committee for Standards in Haematology (BCSH) by the Haemato-oncology sounding board of the British Society for Haematology (BSH). This consists of 50 or more members of the BSH who have reviewed this Guidance and commented on its content and applicability in the UK setting. It has also been reviewed by a patient representative nominated by the Lymphoma Association, but the Association does not necessarily approve or endorse the contents.
Recommendation gradingThe Grading of Recommendations Assessment, Development and Evaluation (GRADE) nomenclature was used to evaluate levels of evidence and to assess the strength of recommendations. The GRADE criteria are specified on the BCSH web site.
BackgroundThis is a new evidence-based guideline on behalf of the BCSH for the management of diffuse large B-cell non-Hodgkin lymphoma following a primary systematic review of the evidence by the writing group using the methodology described above.Diffuse large B-cell lymphoma is the most common non-Hodgkin lymphoma (NHL), accounting for 30-40% of all cases (Rodriguez-Abreu et al, 2007). Although most patients are cured with 6-8 cycles of R-CHOP (rituximab with cyclophosphamide, doxorubicin, vincristine and prednisolone) chemotherapy, about 10-15% have primary refractory disease and a further 20-30% relapse. The
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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.
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