Summary
Mastocytosis is a heterogeneous group of diseases characterized by abnormal proliferation of mast cells. Systemic mastocytosis (SM), in which abnormal mast cells are present in tissues beyond ...the skin, is divided into seven subcategories with varying degrees of severity and prognosis. Very little is known about the epidemiology of SM and its subcategories. This retrospective cohort study of 548 adults with SM diagnosed 1997–2010 was constructed using linked Danish national health registries. The most common subtype of mastocytosis was indolent SM (including urticaria pigmentosa) (n = 450; 82%), followed by SM with subtype unknown (n = 61; 11%), SM with associated clonal haematological non‐mast cell lineage disease (n = 24; 4%), aggressive SM (n = 8; 2%), and mast cell leukaemia (n = 5; 1%). The incidence rate for SM (all subtypes including urticaria pigmentosa) was 0·89 per 100 000 per year. Cumulative incidence was 12·46 per 100 000, and the 14‐year limited‐duration prevalence as of 1 January, 2011 was 9·59 per 100 000. This nationwide cohort from Denmark is the first population‐based epidemiological study of mastocytosis. In this cohort of patients aged 15 years and older, SM was found to be overall relatively rare with notable variation by subtype for patient characteristics, survival and epidemiological measures.
It looks like childhood eczema but is it? Mortz, Charlotte G.; Brockow, Knut; Bindslev‐Jensen, Carsten ...
Clinical and experimental allergy,
June 2019, 2019-06-00, 20190601, Letnik:
49, Številka:
6
Journal Article
Recenzirano
Odprti dostop
In childhood, the most common type of eczema/dermatitis is atopic dermatitis, which occurs in up to 25% of children world‐wide. However, the diagnosis may sometimes be challenging and atopic ...dermatitis may resemble other types of dermatitis as well as other skin diseases such as psoriasis, infections, infestations and malignancies as well as metabolic, genetic and autoimmune disorders. This review will focus on how to recognize the most common types of dermatitis in children and adolescents and how to separate them from the most common differential diagnoses clinically and histologically.
Aim
To investigate and gain knowledge about patients’ perspectives on everyday life with mastocytosis and how they experience, understand and manage symptoms and challenges.
Background
Indolent ...systemic mastocytosis (ISM) is a disease characterised by the accumulation and activation of mast cells. Symptoms are diverse and range from mild to severely debilitating or even fatal. It is considered rare but is underdiagnosed due to lack of awareness. Quantitative studies have shown that ISM can negatively impact quality of life. No qualitative studies have described everyday life with the disease.
Design
A qualitative interview study taking a phenomenological approach.
Methods
Seven qualitative, semi‐structured interviews with adult patients with ISM. The analysis was inspired by Giorgi's phenomenological method. COREQ reporting guidelines were used.
Results
Three themes and five subthemes emerged from the analysis. (a) The everyday life with a rare disease, unknown to most people. Being perceived as a hypochondriac in the encounter with the health system. The diagnosis makes a difference. Expert knowledge is important. (b) Living with and handling the invisible and visible symptoms. The visible body. (c) Fearing an attack. Feeling safe and vulnerable at the same time.
Conclusion
Patients with ISM are severely affected in their everyday lives, especially in terms of their relationship with family and social network. Symptoms restrict and complicate activities and participation in social contexts, and the fear of an anaphylactic attack is always present. The disease affects patients’ self‐perception and sexuality. The rarity of the disease and general low awareness seems to be of great importance in the encounter with the healthcare system, both before and after diagnosis, and there is a need for expert knowledge, support and care.
Relevance for clinical practice
The focus of counselling should not only be on the disease itself, but also on living life with the disease.
Background
While much is known on childhood atopic diseases, knowledge about persistence of atopic diseases from childhood to adulthood is limited. We therefore aimed to study the clinical course of ...atopic diseases and type I sensitization prospectively in an unselected cohort of adolescents followed into adulthood.
Methods
A cohort of unselected 8th‐grade school children (mean age 14 years) established in 1995 and followed up in 2010 were evaluated with questionnaire, clinical examination, skin prick tests and measurements of specific IgE.
Results
The lifetime prevalence of atopic diseases was high and increased significantly from adolescence (31%) to adulthood (57%); particularly allergic rhinitis increased with an incidence rate of 17.5/1000 person‐years. Childhood predictors for adult allergic rhinitis were atopic dermatitis, asthma and asymptomatic sensitization to pollen and house dust mite. Among those with asymptomatic sensitization in adolescence, 53%‐78% developed allergic rhinitis in adulthood. Furthermore, type I sensitization increased significantly from adolescence to adulthood mostly due to increased sensitization to pollen. Type I sensitization was found mainly in those with allergic rhinitis. A high number of adults had oral allergy symptoms due to the high number of birch pollen allergic individuals.
Conclusion
Persistence of atopic diseases in adulthood is common, and a high proportion of the adult population is sensitized giving a high prevalence of allergic rhinitis. Many with asymptomatic sensitization in adolescence will develop allergic rhinitis in adult life. The focus should be on prevention of atopic diseases and sensitization already in childhood.
The lifetime prevalence of allergic rhinitis increased significantly from adolescence to adulthood. Childhood predictors for adult allergic rhinitis were atopic dermatitis and asthma. Furthermore, more than half with asymptomatic sensitization to pollen and house dust mite in adolescence developed allergic rhinitis in adult life. Type I sensitisation increased significantly from adolescence to adulthood mostly due to increased sensitization to pollen.
TABLE 2 Patients with PEG allergy and polysorbate allergy/possible polysorbate allergy ID Elicitor WAO grade Positive SPT (primary evaluation, re-evaluation) Positive BaHR (primary evaluation, ...re-evaluation) Specific IgE (PEG 2000/10,000) Challenge PEG 3350 Primary vaccine offered Secondary vaccine offered Result of vaccination 12–38 M Movicol laxative (PEG 3350) Infliximab (polysorbate 80) ×2 WAO 5 DMG-PEG 2000, PEG 3350, PEG 6000, PEG 20,000 (0.01%), Polysorbate 80 - 0.11/0.63 ND None Comirnaty® divided in four doses Successfully vaccinated ×2 14–31 F Moxaxole laxative (PEG 3350) Brintellix (PEG 400) WAO 2 PEG 2000, DMG-PEG 2000, PEG 3350, PEG 6000, PEG 20,000 (0.01%), Poloxamer 407, Polysorbate 20, Polysorbate 80 - <0.10 ND None Comirnaty® divided in four doses Successfully vaccinated ×3 16–19 F Vaxzevria® vaccine (polysorbat 80) Movicol laxative (PEG 3350) WAO 1 PEG 300, PEG 2000, DMG-PEG 2000, PEG 3000, PEG 6000, PEG 20,000 (0.1%), Poloxamer 407, Polysorbate 20, Polysorbate 80, Spikevax®, Vaxzevria® Spikevax®, Vaxzevria®, Comirnaty® <0.10 Positive None Comirnaty® divided in four doses (Omalizumab treatment—CSU) Successfully vaccinated ×2 18–22 F Primcillin (PEG 6000) Depo-Medrol steroid injection (PEG 3350) WAO 3 PEG 2000, DMG-PEG 2000, PEG 3000, PEG 3350, PEG 6000, PEG 20,000 (0.01%), Poloxamer 407, Polysorbat 80, Polysorbat 20 Poloxamer 407, Comirnaty®, Spikevax® 1.24/2.11 ND None Comirnaty® divided in four doses Successfully vaccinated ×2 10–47 F Depo-Medrol steroid injection (PEG 3350) WAO 1 DMG-PEG 2000, ALC-PEG 2000, PEG 6000, PEG 20,000 (0.1%), Poloxamer 407, Polysorbate 80 DMG-PEG 2000, ALC-PEG 2000, Comirnaty®, Spikevax® 0.27/0.53 ND Comirnaty® ×2 before referral Comirnaty® divided in four doses Successfully re-vaccinated ×1 8–50 F Diprospan steroid injection (PEG 3350) Movicol laxative (PEG 3350) Vagifem (PEG 6000) WAO 1 PEG 2000, DMG-PEG 2000, PEG 3000, PEG 3350, PEG 6000, PEG 20,000 (0.01%), Poloxamer 407, Polysorbat 80 PEG 20,000 (10%), Comirnaty®, Spikevax® 22.70/39.10 ND None Comirnaty® divided in four doses Abstained Anaphylaxis to PEG is rare. 1 Most reactions described in the literature are elicited by depot-steroid injections and laxatives. 1,2 In relation to COVID-19 mRNA vaccines, PEG was identified as a possible elicitor in case of anaphylaxis, since modified PEG 2000 are used as excipient in mRNA vaccines. 3,4 Polysorbate 80 is used in viral vector vaccines and a potential cross-reactivity between PEG and polysorbate has been hypothesized, 2 due to structural relationship. Why these PEG allergic patients tolerated the vaccine with modified PEG 2000 may be explained by the patients individual threshold in relation to the small amount of PEG in the mRNA vaccine, the lower molecular weight of PEG 2000 compared to higher molecular weight PEGs in for example, laxatives and depot-steroids (PEG 3350), or the conjugation with lipid-nanoparticles in mRNA vaccines. 11,12 Titrated vaccination has been described in PEG allergic patients. CONFLICTS OF INTEREST The authors report no potential conflict of interest.
Platelet-activating factor (PAF) is an important proinflammatory factor produced and secreted by several types of cells, including mast cells, monocytes, tissue macrophages, platelets, eosinophils, ...endothelial cells, and neutrophils, and is involved in allergic inflammation.5 In this letter, we emphasize the influence of PAF on skin barrier and KC TJ integrity in AD and further analyze TJ disruption in diseased skin for better understanding the molecular and cellular mechanisms in the regulation of TJ barrier integrity. Slides were mounted in ProLong-Gold with 4'-6-diamidino-2-phenylindole, dihydrochloride (Invitrogen-Thermofisher) and confocal images were collected using a Leica TCS SPE confocal microscope (Leica Microsystems, Heerbrugg, Switzerland). mRNA analysis by Taqman low-density array microfluidic cards We chose 62 predesigned probes representing the junctional apparatus of epithelial cells from the Applied Biosystem database.
Mastocytosis is a term used to denote a heterogeneous group of conditions defined by the expansion and accumulation of clonal (neoplastic) tissue mast cells in various organs. The classification of ...the World Health Organization (WHO) divides the disease into cutaneous mastocytosis, systemic mastocytosis, and localized mast cell tumors. On the basis of histomorphologic criteria, clinical parameters, and organ involvement, systemic mastocytosis is further divided into indolent systemic mastocytosis and advanced systemic mastocytosis variants, including aggressive systemic mastocytosis and mast cell leukemia. The clinical impact and prognostic value of this classification has been confirmed in numerous studies, and its basic concept remains valid. However, refinements have recently been proposed by the consensus group, the WHO, and the European Competence Network on Mastocytosis. In addition, new treatment options are available for patients with advanced systemic mastocytosis, including allogeneic hematopoietic stem cell transplantation and multikinase inhibitors directed against KIT D816V and other key signaling molecules. Our current article provides an overview of recent advances in the field of mastocytosis, with emphasis on classification, prognostication, and emerging new treatment options in advanced systemic mastocytosis.
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