The role of the clinical and psychological symptoms of mastocytosis in the patient’s quality of life

Spolak-Bobryk, Natalia; Niedoszytko, Marek; Jassem, Ewa; Chełmińska, Marta; Lange, Magdalena; Majkowicz, Mikołaj; Nedoszytko, Bogusław; Borchet, Judyta

doi:10.5114/ada.2021.108433

Cited by 7 publications

(11 citation statements)

References 30 publications

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“…The frequency of anxiety, high stress and irritability were similarly high among mastocytosis patients. Anxiety was reported in up to 27% of patients, 40 stress in 42%–46% 14,30 and irritability in 54% 9 . Wide mood fluctuatioikns were also especially prevalent, with anger and fatigue being the most frequent manifestations 27 .…”

Section: Resultsmentioning

confidence: 99%

“…Neuropsychiatric evaluations encompassed a range of assessments, from psychiatric interviews 23,27,31 to depression questionnaires 9,10,14,15,22–24,30 and quality of life 9,22,23 scores. The MC Quality of Life Questionnaire 43 was conducted only in one of the studies 40 . Cognitive tests focused on memory 24,27 and CI 13,27 .…”

Section: Resultsmentioning

confidence: 99%

“…9,14,15 In contrast, the lifetime frequency of depression in the general population varies between 2% and 21%, with the highest rates found in some European countries and the lowest in some Asian countries. 46 Concurrently, anxiety was reported in up to 27% of mastocytosis patients, 40 stress in 42%-46% 14,30 and irritability in 54%. 9 Anxiety is frequently related to the fear of suffering anaphylactic reactions outdoors following insects' stings, which can limit and complicate outdoor activities and social participation.…”

Section: Discussionmentioning

confidence: 99%

“…Concurrently, anxiety was reported in up to 27% of mastocytosis patients, 40 stress in 42%–46% 14,30 and irritability in 54% 9 . Anxiety is frequently related to the fear of suffering anaphylactic reactions outdoors following insects' stings, which can limit and complicate outdoor activities and social participation 40,47 .…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

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

Sagües‐Sesé,

García‐Casares,

Álvarez‐Twose

2023

Clinical & Translational All

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“…A high baseline tryptase level at least 100 ng/ml correlates with disease progression or a developing myeloid/lymphoid neoplasia [36]. Adult mastocytosis patients experience decreased quality of life and anxiety which correlates with allergic symptoms including anaphylaxis [37]. The prevalence of anaphylaxis or neurocognitive dysfunction is not increased in paediatric MPCM [22].…”

Section: Prognosis and Follow-up In Paediatric Mastocytosismentioning

confidence: 99%

Updates in diagnosis and management of paediatric mastocytosis

Tiano

Krase

Sacco

2022

Current Opinion in Allergy &Amp; Clinical Immunology

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Purpose of reviewPaediatric mastocytosis is a rare clonal disorder characterized by the overproduction and organ infiltration of mast cells. Symptoms are due to mast cell mediator release. Cutaneous mastocytosis is the most common presentation in children with systemic disease being rare. Our aim is to provide a practical guideline in differentiating subtypes of paediatric mastocytosis while providing actionable recommendations on diagnosis, clinical management, follow-up and prognosis. Recent findingsLongitudinal cohort studies of paediatric cutaneous mastocytosis have shown spontaneous remission with favourable prognosis. Hereditary alpha-tryptasemia may coexist with mastocytosis; thus, screening for this disorder is recommended. There is an emerging role for serum tryptase in asthma endotyping and potential for using therapeutic tryptase inhibitors. SummaryMorbidity in paediatric mastocytosis typically arises from symptoms secondary to mast cell mediator release. Prognosis for nonaggressive disease is typically favourable; however, risks for anaphylaxis and psychosocial morbidity may be underestimated. Symptomatic management and anticipatory guidance may help support patients and families throughout the disease course.

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A cross‐sectional study on quality of life in adult indolent systemic mastocytosis and its association with cutaneous involvement

Isoletta,

De Silvestri,

Bonelli

et al. 2024

JEADV Clinical Practice

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BackgroundSystemic mastocytosis (SM) is characterized by abnormal mast cell accumulation in various tissues, including the skin, and encompasses several clinical variants, ranging from less aggressive to more severe forms. ISM, the most common variant, often presents with cutaneous manifestations, causing significant impairment in QoL due to mediator‐release symptoms and stigma associated with typical cutaneous lesions.ObjectivesThe study aimed to assess QoL impairment in ISM patients and its correlation with demographic and clinical data.MethodsA cohort of 52 adult ISM patients was evaluated using the Dermatology Life Quality Index (DLQI) and the mastocytosis QoL questionnaire (MC‐QoL). Questionnaire scores were then analyzed in relation to the clinical and demographic characteristics of the patients.ResultsOver half of the patients experienced mild or higher levels of impairment in QoL, with female patients showing greater impairment than males. Patients with recent symptom onset reported higher MC‐QoL scores, possibly due to psychological factors and lack of disease knowledge. Cutaneous symptoms significantly impacted QoL, and the visibility of lesions affected DLQI scores more than MC‐QoL scores. A moderately strong correlation was observed between DLQI and MC‐QoL scores, particularly in the Skin and Social Life domains. The study highlights gender differences in QoL impairment, suggesting the need for further investigation into potential social or cultural factors.ConclusionsWhile the study provides valuable insights into QoL impairment in ISM patients, its monocentric nature and small sample size are notable limitations. Further research is warranted to better understand the impact of SM on patients' well‐being and to identify effective strategies for managing symptoms and improving overall QoL in this population.

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The role of the clinical and psychological symptoms of mastocytosis in the patient’s quality of life

Cited by 7 publications

References 30 publications

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

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

Updates in diagnosis and management of paediatric mastocytosis

A cross‐sectional study on quality of life in adult indolent systemic mastocytosis and its association with cutaneous involvement

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