Development and validation of the mastocytosis quality of life questionnaire: MC-QoL

Siebenhaar, Frank; Tschirnhaus, Elisabeth von; Hartmann, Karin; Rabenhorst, Anja; Staubach, Petra; Peveling‐Oberhag, Adriane; Wagner, Nicola; Martus, Peter; Carter, M. C.; Metcalfe, Dean D.; Church, Martin K.; Maurer, Marcus; Weller, Karsten

doi:10.1111/all.12842

Cited by 51 publications

(46 citation statements)

References 33 publications

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“…Research in this field is currently hampered by the lack of appropriate and validated PRO instruments . We recently reported the development and validation of the Mastocytosis Quality of Life questionnaire (MC‐QoL), a disease‐specific tool to measure HRQoL impairment in patients with CM and ISM . Here, we report the development and validation of the MAS, a 9 item PRO instrument for the assessment of disease activity in patients with mastocytosis.…”

Section: Discussionmentioning

confidence: 99%

“…To identify and generate potential items for the MAS, a combined approach of three strategies was applied as follows: (i) exploratory semi‐structured interviews with 10 patients suffering from CM (n = 3) or ISM (n = 7). Interviews also served for the item generation of the Mastocytosis Quality of Life Questionnaire MC‐QoL, which was developed and validated in a parallel study . (ii) A literature search for published data on mastocytosis‐related symptoms and (iii) mastocytosis expert opinions on potentially relevant items.…”

Section: Methodsmentioning

confidence: 99%

“…Item selection (reduction) was performed by impact analysis in combination with a review of its results for face validity (content validity). As for the development of MC‐QoL, all signs and symptoms identified in the item generation phase were administered to 76 mastocytosis patients (55 females; mean age ± SD: 51.6 ± 13.5 years, median: 52.5, range: 20‐77) recruited from the outpatient clinic at the Department of Dermatology and Allergy, Charité — Universitätsmedizin Berlin, Germany . The patients completed all potential MAS items and subsequently evaluated their relevance and importance (impact analysis).…”

Section: Methodsmentioning

confidence: 99%

“…MC‐QoL is a mastocytosis‐specific QoL instrument consisting of 27 items in 4 domains (“symptoms,” “functioning/social life,” “emotions” and “skin”) with a recall period of 2 weeks. MC‐QoL can be computed as domain scores or total score, each ranging from 0 to 100 points, with higher scores indicating a higher impairment of life quality …”

Section: Methodsmentioning

confidence: 99%

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Development and validation of the mastocytosis activity score

Siebenhaar

Sander

et al. 2018

Allergy

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

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Development and validation of the mastocytosis activity score

Siebenhaar

Sander

et al. 2018

Allergy

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“…Given the heterogeneous presentation, patients may experience little or no inconvenience related to their condition, or they may suffer from symptoms with a high impact on their quality of life (QoL). Recent studies have validated several QoL questionnaires for mastocytosis, identifying fatigue and fear of anaphylaxis as the symptoms causing the greatest inconvenience and impact on patient QoL [13•, 14•].…”

Section: Presentationmentioning

confidence: 99%

Mast Cell Clonal Disorders: Classification, Diagnosis and Management

Onnes

Tanno

Elberink

2016

Curr Treat Options Allergy

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Mast cell clonal disorders are characterized by the clonal proliferation of pathological mast cells as a result of somatic mutations in the KIT gene, most commonly the D816V mutation. Accumulation and degranulation of these cells causes a wide variety of symptoms. Mast cell clonal disorders can be divided into mastocytosis and monoclonal mast cell activation syndrome, depending of the level of clonality. The severity of mastocytosis varies from an indolent variant with a good prognosis, to an aggressive condition with short life expectancy. Diagnosis is based on demonstration of clonality and accumulation in the skin and in extracutaneous tissues. Treatment is highly individualized, and is based on the severity of the condition. Treatment of patients with indolent systemic mastocytosis is aimed at reducing symptoms, using histamine H1 and H2 receptor antagonists as a starting point. In addition, associated conditions such as osteoporosis must be treated. Treatment of advanced systemic mastocytosis is aimed at reducing mast cell load through cytoreductive therapy. The choice of such therapy depends on the KIT mutational status. Though currently there is no curative treatment available, promising new therapies such as midostaurin are emerging that have demonstrated success in reducing symptoms and improving quality of life.

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Cognitive, neuropsychiatric and neurological alterations in mastocytosis: A systematic review

Sagües‐Sesé,

García‐Casares,

Álvarez‐Twose

2023

Clinical & Translational All

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BackgroundMastocytosis manifests with multisystemic symptoms, often involving the nervous system. Numerous cognitive, neuropsychiatric and neurological alterations have been reported in multiple observational studies.MethodsWe performed a qualitative systematic literature review of reported data consulting the electronic databases Medline, Scopus, Web of Science, Cochrane, and BASE until June 2023.ResultsWe selected 24 studies in which the majority showed that a high proportion of mastocytosis patients suffer cognitive, neuropsychiatric and neurological alterations. The most common disorders and estimated ranges of frequency observed in adults were depression (68%–75%), anxiety, high stress or irritability (27%–54%), cognitive impairment (27%–39%, primarily affecting memory skills), and headaches (55%–69%). Attention challenges and learning difficulties were reported in children at a rate of 13%, while neurodevelopmental disorders occurred at rates of 8%–12%. Frequent white abnormalities in mastocytosis patients with concomitant psychocognitive symptoms have been reported although neuroimaging studies have been performed rarely in this population.ConclusionFurther studies with more comprehensive and homogeneous evaluations and neuroimaging and histological analysis should be performed for a better understanding of these manifestations. An earlier detection and proper management of these symptoms could greatly improve the quality of life of these patients.

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Development and validation of the mastocytosis quality of life questionnaire: MC-QoL

Abstract: The MC-QoL is the first disease-specific HRQoL questionnaire for adult patients with cutaneous and indolent systemic mastocytosis. This short, validated and reliable instrument will serve as a valuable tool in future clinical studies and in routine patient care.

Cited by 51 publications

References 33 publications

Development and validation of the mastocytosis activity score

Development and validation of the mastocytosis activity score

Mast Cell Clonal Disorders: Classification, Diagnosis and Management

Cognitive, neuropsychiatric and neurological alterations in mastocytosis: A systematic review

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