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Background There is need for an accurate diagnostic test in mastocytosis patients with wasp venom allergy (WVA) and monitoring of these patients during immunotherapy (IT). In this study, we aimed to evaluate sensitivity and specificity of the Basophil Activation Test (BAT) as a diagnostic and monitoring test in patients with mastocytosis and WVA. Methods Seventeen patients with mastocytosis and WVA and six mastocytosis patients without WVA were included. BAT was performed before the start of IT (first visit) and at 6 weeks (second visit) and 1 year (third visit), after reaching the maintenance dose. Of 17 patients included, 11 completed the third visit. In mastocytosis patients with WVA, dose-dependent wasp-venom induced upregulation of CD63 and CD203c expression on basophils was observed compared with mastocytosis patients without WVA. Serum specific IgE, IgG4, and tryptase levels were measured in all patients. Results BAT had a sensitivity of 87% and specificity of 100% in diagnosing WVA in mastocytosis patients. Basophil allergen threshold sensitivity with respect to CD63 and CD203c was significantly decreased in the second visit compared with the first visit and increased significantly in the third visit compared with the second visit. Specific IgE levels increased significantly in the second visit compared with first and decreased significantly in the third visit compared with the second. Specific IgG4 levels rose significantly in the second visit compared with the first and on the third visit compared with the second. Tryptase levels did not change significantly during the study. Conclusions BAT represents a diagnostic test with 100% specificity in allergic patients with mastocytosis and these patients are better to be monitored for a longer period during IT. (c) 2013 International Clinical Cytometry Society

P>Background: Anaphylaxis to insect venom (Hymenoptera) is most severe in patients with mastocytosis and may even lead to death. However, not all patients with mastocytosis suffer from anaphylaxis. The aim of the study was to analyze differences in gene expression between patients with indolent systemic mastocytosis (ISM) and a history of insect venom anaphylaxis (IVA) compared to those patients without a history of anaphylaxis, and to determine the predictive use of gene expression profiling. Methods: Whole-genome gene expression analysis was performed in peripheral blood cells. Results: Twenty-two adults with ISM were included: 12 with a history of IVA and 10 without a history of anaphylaxis of any kind. Significant differences in single gene expression corrected for multiple testing were found for 104 transcripts (P

BACKGROUND: Mastocytosis is an uncommon disease resulting from proliferation of abnormal mast cells infiltrating skin, bone marrow, liver, and other tissues. The aim of this study was to find differences in gene expression in peripheral blood cells of patients with indolent systemic mastocytosis compared to healthy controls. The second aim was to define a specific gene expression profile in patients with mastocytosis. METHODS: Twenty-two patients with indolent systemic mastocytosis and 43 healthy controls were studied. Whole genome gene expression analysis was performed on RNA samples isolated from the peripheral blood. For amplification and labelling of the RNA, the Illumina TotalPrep 96 RNA Amplification Kit was used. Human HT-12_V3_expression arrays were processed. Data analysis was performed using GeneSpring, Genecodis, and Transcriptional System Regulators. RESULTS: Comparison of gene expression between patients and controls revealed a significant difference (P 2 in gene expression in 2303 of the 48.794 analysed transcripts. Functional annotation indicated that the main pathways in which the differently expressed genes were involved are ubiquitin-mediated proteolysis, MAPK signalling pathway, pathways in cancer, and Jak-STAT signalling. The expression distributions for both groups did not overlap at all, indicating that many genes are highly differentially expressed in both groups. CONCLUSION: We were able to find abnormalities in gene expression in peripheral blood cells of patients with indolent systemic mastocytosis and to construct a gene expression profile which may be useful in clinical practice to predict the presence of mastocytosis and in further research of novel drugs.

A 57-year-old woman with advanced non-small cell lung carcinoma developed hypersensitivity reactions to docetaxel. Measures taken to attempt the re-administration of docetaxel failed. For the differential diagnosis, an IgE specific to docetaxel (in terms of cross-reactivity with Taxus baccata), the solubilizing agent polysorbate 80, as well as the possibility of the reaction being non-IgE-mediated, were all considered. The latter was thought to be most likely. Desensitisation has been reported to be safe and effective in protecting patients from severe hypersensitivity reactions in both IgE- and non-IgE-mediated reactions. Desensitisation in this context means the induction of temporary clinical unresponsiveness to the culprit drug. The gradual reintroduction of small doses of the drug at fixed time intervals eventually allows delivery of full therapeutic doses. Desensitisation to docetaxel was successfully carried out in a supervised setting a total of three times in this patient.

P>Background: Health-related quality of life (HRQL) has never been measured with both generic and disease-specific questionnaires in the same group of food allergic patients. The aim of this study was to compare HRQL of food allergic patients as measured with generic and disease-specific questionnaires. Methods: Generic questionnaires (CHQ-CF87 and RAND-36) and disease-specific HRQL questionnaires (FAQLQ-CF, -TF and -AF) were completed by 79 children, 74 adolescents and 72 adults with food allergy. Floor and ceiling effects, percentage of agreement and multivariate stepwise regression analysis were used to compare the generic and disease-specific measurements. Results: The Food Allergy Quality of Life Questionnaires (FAQLQs) showed minimal floor or ceiling effects. The CHQ-CF87 and RAND-36 showed minimal floor effects, but remarkable ceiling effects (> 73%) were found for the scales role functioning-emotional (RE), role functioning-behaviour (RB), role functioning-physical (RP) in children and adolescents and the scale RE (> 79%) in adults. Additionally, we found low percentages of agreement between the generic and disease-specific questionnaires to identify the same food allergic patients with the best or worst HRQL. Only patients with the best disease-specific HRQL also tended to have the best generic HRQL. Finally, the explained variance in HRQL by patient characteristics was higher in the disease-specific questionnaires (30.7-62.8%) than in the generic scales (6.7-31.7%). Conclusion: Disease-specific HRQL questionnaires may be more suitable to measure clinically important impairments in HRQL or HRQL differences over time in food allergic patients. However, generic HRQL questionnaires are indispensable for the comparison between different diseases and are thus complementary.

Background: The vast majority of children will undergo their first exposure to common allergenic foods at home. However, the first exposure may lead to clinical reactions. It has been proposed to introduce allergenic foods gradually into the diets of children at risk for food allergy, but no practical dietary advice has been devised. Objective: The aim of this study was to devise safe introduction schedules for common allergenic foods for use at home, based on the challenge doses as administered in double-blind, placebo-controlled food challenge (DBPCFCs) in children who were never exposed previously to these foods. Methods: Seventy-two DBPCFCs were performed in 63 children as a first known exposure. The incrementing challenge doses were converted into equivalent portions of these foods in their usual household form and incorporated in introduction schedules. The feasibility of the introduction scales was tested in parents of the children attending our clinic. Results: Based on the results of the positive challenges (37) in which severe reactions did not occur, detailed introduction schedules and a reference photograph of the required increasing amounts of food were devised for use at home. Feasibility testing showed that, when using these introduction schedules, parents portioned the initial doses significantly lower than without detailed instructions. Conclusions: The introduction schedules and reference photograph provide information for parents to introduce the required amounts of allergenic foods in initial low doses at home. This is expected to improve the safety of this procedure.

Purpose of review Until recently, quality-of-life measures were only used in allergic diseases wit ongoing symptoms, such as asthma and rhinoconjunctivitis. Anaphylaxis is a chronic disease without ongoing physical symptoms, but the problems concerning quality of life are related to the continuous vigilance required to prevent accidental exposure. This raises specific issues concerning the validation of quality-of-life instruments. Recent findings The preferred independent measure for validation generally is an objective measurement of the severity of disease (e.g. spirometry in asthma). In patients suffering from anaphylaxis, the perceived expectation of what will happen following exposure can be used as the key independent measure. Recently, a specific instrument measuring this expectation (the 'Expectation of outcome' questionnaire) has been developed, and successfully used in insect-venom anaphylaxis. Summary Also in diseases without ongoing symptoms like anaphylaxis, quality of life can be measured and the disease-specific instrument validated. It is to be expected that many new instruments will be developed in the coming years to address important issues in anaphylaxis. They may provide a better understanding of the major problems of certain patient subgroups and may give direction to the kind of information that should be addressed and what kind of interventions could be important and whether they are useful or not.

Background: Diagnosis and management of allergic patients is often initially and exclusively performed by GPs. Since the prevalence of allergy is increasing and many GPs report having difficulties in diagnosing and managing allergic patients, an Allergy Management Support System (AMSS) for primary care is under development. The aim of this study was to determine the agreement on allergy management between the AMSS and allergy specialists. Methods: The questionnaire of the AMSS was administered to patients suspected of being allergic who were referred by GPs to (pediatric) allergists or dermatologists. The responses were analyzed by 2 researchers who allocated the patients into predefined management categories using the algorithms of the AMSS. Based on the clinical correspondence, 2 allergists and 2 dermatologist allocated the same patients in the same way using their clinical knowledge, which was considered to be the gold standard. Agreement between the gold standard and the AMSS was investigated by ascertaining concordance of management categories. The inter-rater reliability between the specialists and researchers was calculated using Cohen's kappa. Results: 42 patients (38% male, mean age 26.2 years) completed the questionnaire. Mean time between completing the questionnaire and the consult with allergy specialist was 8 days (SD 11 days). Agreement between the gold standard and the AMSS was found in 69.2% (CI 67.2-71.2) of cases. There was moderate agreement between the allergists (k = 0.55, P