Cow's milk allergy can be monitored through the degree of competition between specific IgE and IgG

European-Academy-of-Allergology-and-Clinical-Immunology Digital Congress (EAACI), London, ENGLAND, JUN 06-08, 202

Higher prevalence of vertebral fractures in systemic mastocytosis, but not in cutaneous mastocytosis and idiopathic mast cell activation syndrome

International audienceLittle is known about osteoporosis in mast cell disorders (MCDs) not related to systemic mastocytosis. We described osteoporosis and fractures in MCDs and showed that systemic mastocytosis was the only studied MCDs associated with osteoporotic vertebral fractures

Meeting Abstract: PD0389

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Photo-induced graft-versus-host disease

International audienceOverlap chronic graft-versus-host disease (GVHD) associates both features of acute and chronic GVHD. Trigger factors for chronic GVHD are unclear. We describe two patients who received allogenic haematopoietic stem-cell transplantation, and who later developed overlap chronic GVHD after sun exposure. Available data from in vivo investigations suggest ultraviolet B radiation (UVB) has a beneficial effect on acute and chronic GVHD. The role of sun irradiation as a trigger for isomorphic cutaneous GVHD has been rarely reported in the literature. Herein, we demonstrate for the first time, using repetitive broadband phototesting, that UVB triggers chronic GVHD

Antigen 5-spiked Vespula and Polistes venom extracts for Vespid allergy diagnostics: A French multicenter study

International audienceVespula and Polistes spp venom extracts (VEs) for ImmunoCAP (Thermo Fisher Scientific, Uppsala, Sweden) platforms have beenspiked, that is, enriched, with group 5 allergens since 2012. Limited information was available from the manufacturer, but small cohortshave reported altered performance for in vitro diagnostics.1,2 Herein we present the results of a French multicenter study onantigen 5–spiked Vespula and Polistes VEs compared with nonspiked VE

Telangiectasia macularis eruptiva perstans (TMEP): A form of cutaneous mastocytosis with potential systemic involvement

International audienceBackground: Telangiectasia macularis eruptiva perstans (TMEP) has not been fully characterized.Objective: We sought to estimate the frequency and clinical characteristics of TMEP in a cohort of adult patients with cutaneous mastocytosis, and to assess the presence of systemic involvement.Methods: We included all consecutive patients evaluated for cutaneous mastocytosis in 2 centers: the Mastocytosis Competence Center of the Midi-Pyrénées from May 2006 to December 2013, and the French Reference Center for Mastocytosis from January 2008 to September 2013. Skin phenotype, histopathology, presence of KIT mutation in the skin, and assessment of systemic involvement according to World Health Organization (WHO) criteria were prospectively investigated.Results: Of 243 patients with cutaneous mastocytosis, 34 (14%) were given a diagnosis of TMEP. The diagnosis of systemic mastocytosis was established in 16 patients (47%) with TMEP. Three patients (9%) had aggressive systemic mastocytosis (C-findings according to WHO). In all, 32 patients (94%) exhibited at least 1 mast cell activation-related symptom.Limitations: Patient recruitment was undertaken at 2 referral centers with expertise in the diagnosis and treatment of mastocytosis so that the clinical findings and incidence of systemic involvement may be overestimated in comparison with the overall population of patients with TMEP.Conclusion: TMEP accounts for about 14% of patients with cutaneous mastocytosis. The disease manifests as mast cell activation symptoms in almost all patients and can be associated with systemic involvement in about 50% of cases

Bone marrow tryptase as a possible diagnostic criterion for adult systemic mastocytosis

International audienceBackground: Mastocytosis is difficult to diagnose, especially when systemic mast cell activation symptoms are not present or involve only one extracutaneous organ.Objective: The main objective was to evaluate the accuracy of the bone marrow tryptase level in the diagnosis of systemic mastocytosis in patients with a clinical suspicion of mastocytosis.Methods: We included all adult patients evaluated in our centre between December 2009 and 2013 for suspected mastocytosis as part of a standardized procedure and who had a bone marrow and serum tryptase assay on the same day. The diagnosis of systemic mastocytosis was established on the basis of the World Health Organization criteria as the gold standard. The accuracy of the bone marrow tryptase level in the diagnosis of systemic mastocytosis was assessed by a receiver operating characteristics curve analysis. The different sensitivity and specificity values, corresponding to the set of possible bone marrow tryptase level cut-off values, were estimated with 95% confidence intervals.Results: Seventy-three patients were included. The diagnosis of systemic mastocytosis was established in 43 patients (58.9%). The median bone marrow tryptase level was 423 μg/L [95% CI: 217-868] in the systemic mastocytosis group and 7.5 μg/L [95% CI: 4.6-17.1] in the non-systemic mastocytosis group (P < 0.001). A cut-off value of 50 μg/L for bone marrow tryptase identified systemic mastocytosis with a sensitivity of 93.0% [95% CI: 80.9-98.5%] and a specificity of 90.0% [95% CI: 73.5-97.9%].Conclusion and clinical relevance: The bone marrow tryptase level appears to be a valuable diagnostic criterion for confirming systemic mastocytosis. If this diagnosis can reliably be excluded by evaluation of the bone marrow tryptase level, there would be no need to perform a bone marrow biopsy

Mast cell activation syndrome: High frequency of skin manifestations and anaphylactic shock

International audienceMast cell activation syndrome (MCAS), is associated with mast cell activation-related symptoms involving two or more organs. The disease in patients with MCAS does not fulfill the diagnostic criteria for systemic (SM) or cutaneous mastocytosis. There is limited information about the clinical and biological characteris-tics of MCAS in clinical practice.1,2,4 The performance of the proposed diagnostic criteria for MCAS has not been prospectively assessed. Bone marrow tryptase has been shown to be a sensitive diagnostic tool for SM even in patients with normal serum tryptase (ST).The primary objective of this study was to describe the clinicaland paraclinical characteristics of a cohort of patients with primary MCAS. The secondary objective was to describe the usability of the two available sets of diagnostic criteria for MCAS. We included all consecutive adult patients with a diagnosis of primary MCAS evaluated between April 2011 and May 2015. The diagnostic of MCAS was established using the diagnostic criteria defined by Valent et al. and/or those defined by Molderings et al. The presence/absence of a monoclonal mast cell carrying the D816V KIT mutation and/or expression of CD25 in bone marrow determined the classification of patients as monoclonal MCAS (MMCAS)/idiopathic MCAS (IMCAS) respectively

Response to commentary by Drs. Poncet and Sénéchal

International audienceWe thank Drs. Poncet and Sénéchal for their interest and critical reading of our paper. We are well aware of their pioneering work on molecular aspects of cypress pollinosis. However, the focus of our paper was rather on epidemiological, clinical and diagnostic features of peach allergy and not towards molecular pollen determinants. We hereby provide answers to direct questions and notions made by Drs. Poncet and Sénéchal. 1. In regard to literature references, we cited those we found relevant for the scope and purpose of the paper and none of the three reviewers suggested citation of additional publications. To our knowledge, the paper by Hugues et al (2006) was indeed the first to report an association between cypress and peach allergy, and it is cited as reference 39 in our paper 1. However, contrary to the assertion by Poncet and Sénéchal, the authors of that paper did not identify a pollen homologue of Pru p 7, which was first reported as an allergen by Tuppo et al in 2013 2 but instead made the notion "Because both allergenic extracts include a 45 kDa-allergen, it should be the shared allergen." Cup a 1, a major allergen in cypress pollen, has a molecular weight of 43 kDa. Experimental data from the Poncet team are currently available for BP14 and snakin-1, neither of which have been officially recognized and named as allergens by the WHO/IUIS Allergen Nomenclature SubCommittee (www.aller gen.org, accessed May 4 2019). Other papers cited by Poncet et al are either replies or reviews. We prefer citation of original, peer-reviewed research. However, the review on cypress pollinosis is also cited in our paper as ref 41. 3 2. This case report of one patient with discordant FABER IgE and BAT results would have brought little if any further information to the reader. In our hands, the FABER test displays highly sensitive detection of IgE to Pru p 7. 3. As noted above, BP14 has not been officially recognized as an allergen. 4. Snakin-1 is out of the scope of our publication. 5. Recombinant Pru p 7 was biochemically and immunologically characterized as described in section 2.6 4 and additionally by circular dichroism spectroscopy, but, given the focus of the paper, we did not consider it relevant to show and elaborate on such data, nor was there space available. It was also not suggested by any of the three reviewers. However, the BAT results shown in Table S3 demonstrate a similar functional potency of natural and recombinant Pru p 7 which suggests an authentic folding of the recombinant protein. o We do not agree that assessment of anti-microbial activity of recombinant Pru p 7 and several of the other specifics mentioned would be necessary to validate the association between cypress pollen allergy and peach allergy as suggested (but not yet done in their publications) by Drs Poncet and Sénéchal. o The cypress species used was Cupressus sempervirens. o The pollen was extracted and clarified by standard methods. We did not consider total protein concentration to be informative in relation to the purpose of the experiment but chose instead to determine the potency of the extract by titrated inhibition of IgE binding to Pru p 7, as described in section 3.5. That potency determination guided the choice of inhibitor concentration in the single-point inhibitions shown in figure 5A. 15% (w/v) means a concentration corresponding to 15 g of pollen (dry weight) per 100 mL of liquid, a manner of expressing concentrations also used by Poncet et al in their papers. o The specificity of inhibition with the pollen extract was ensured by a complete lack of inhibition of binding of dog dander specific IgE to dog dander ImmunoCAP (e5) and is further indicated by the lack of significant inhibition in some samples as shown in figure 5A. Had the inhibitory effect of the cypress pollen extract been due to unspecific blockade of IgE, no such results would have been obtained. We hope that our response will provide sufficient clarity and explanation to the questions raised by Drs. Poncet and Sénéchal