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High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience

Summary Background Systemic mastocytosis (SM) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due...

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Published in:Clinical and experimental allergy 2014-01, Vol.44 (1), p.121-129
Main Authors: Gülen, T., Hägglund, H., Dahlén, B., Nilsson, G.
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Dahlén, B.
Nilsson, G.
description Summary Background Systemic mastocytosis (SM) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release. Objective The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult SM patients. We also explored the clinical spectrum of mast cell mediator‐related symptoms in patients with SM. Methods This descriptive study was performed among 84 consecutive adult (≥ 18 years) patients those were diagnosed with SM according to WHO criteria. Sixty‐six of the patients also underwent a comprehensive allergy work‐up. Results Sixty of 84 patients with SM (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for SM and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty‐six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19/36; 53%). In 39% (14/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with SM, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in SM patients with anaphylaxis as compared with non‐anaphylaxis SM patients, 70% vs. 23%, respectively (P = 0.0002). Conclusions and Clinical Relevance Anaphylaxis is more prevalent in patients with SM, predominantly in patients with atopic SM. Hymenoptera venom‐induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.
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The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release. Objective The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult SM patients. We also explored the clinical spectrum of mast cell mediator‐related symptoms in patients with SM. Methods This descriptive study was performed among 84 consecutive adult (≥ 18 years) patients those were diagnosed with SM according to WHO criteria. Sixty‐six of the patients also underwent a comprehensive allergy work‐up. Results Sixty of 84 patients with SM (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for SM and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty‐six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19/36; 53%). In 39% (14/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with SM, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in SM patients with anaphylaxis as compared with non‐anaphylaxis SM patients, 70% vs. 23%, respectively (P = 0.0002). Conclusions and Clinical Relevance Anaphylaxis is more prevalent in patients with SM, predominantly in patients with atopic SM. Hymenoptera venom‐induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.</description><identifier>ISSN: 0954-7894</identifier><identifier>ISSN: 1365-2222</identifier><identifier>EISSN: 1365-2222</identifier><identifier>DOI: 10.1111/cea.12225</identifier><identifier>PMID: 24164252</identifier><language>eng</language><publisher>England: Blackwell Publishing Ltd</publisher><subject>Adult ; Aged ; Aged, 80 and over ; Allergens - immunology ; Allergies ; anaphylaxis ; Anaphylaxis - complications ; Anaphylaxis - diagnosis ; Anaphylaxis - epidemiology ; Anaphylaxis - etiology ; atopic disease ; Comorbidity ; Female ; Humans ; Hymenoptera ; hymenoptera venom ; IgE sensitization ; Immunoglobulin E - immunology ; Male ; Mast Cells - immunology ; Mast Cells - metabolism ; mastocytosis ; Mastocytosis, Systemic - complications ; Medical research ; Medicin och hälsovetenskap ; Middle Aged ; Prevalence ; Risk Factors ; Skin Tests ; tryptase ; Young Adult</subject><ispartof>Clinical and experimental allergy, 2014-01, Vol.44 (1), p.121-129</ispartof><rights>2013 John Wiley &amp; Sons Ltd</rights><rights>2013 John Wiley &amp; Sons Ltd.</rights><rights>Copyright © 2014 John Wiley &amp; Sons Ltd</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c5495-3998552ec69e39778f65c2ba873714b2dd0e9eca0bea7d1becd541d490dbc77a3</citedby><cites>FETCH-LOGICAL-c5495-3998552ec69e39778f65c2ba873714b2dd0e9eca0bea7d1becd541d490dbc77a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>230,314,780,784,885,4024,27923,27924,27925</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/24164252$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink><backlink>$$Uhttps://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-270848$$DView record from Swedish Publication Index$$Hfree_for_read</backlink><backlink>$$Uhttp://kipublications.ki.se/Default.aspx?queryparsed=id:127937851$$DView record from Swedish Publication Index$$Hfree_for_read</backlink></links><search><creatorcontrib>Gülen, T.</creatorcontrib><creatorcontrib>Hägglund, H.</creatorcontrib><creatorcontrib>Dahlén, B.</creatorcontrib><creatorcontrib>Nilsson, G.</creatorcontrib><title>High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience</title><title>Clinical and experimental allergy</title><addtitle>Clin Exp Allergy</addtitle><description>Summary Background Systemic mastocytosis (SM) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release. Objective The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult SM patients. We also explored the clinical spectrum of mast cell mediator‐related symptoms in patients with SM. Methods This descriptive study was performed among 84 consecutive adult (≥ 18 years) patients those were diagnosed with SM according to WHO criteria. Sixty‐six of the patients also underwent a comprehensive allergy work‐up. Results Sixty of 84 patients with SM (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for SM and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty‐six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19/36; 53%). In 39% (14/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with SM, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in SM patients with anaphylaxis as compared with non‐anaphylaxis SM patients, 70% vs. 23%, respectively (P = 0.0002). Conclusions and Clinical Relevance Anaphylaxis is more prevalent in patients with SM, predominantly in patients with atopic SM. Hymenoptera venom‐induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.</description><subject>Adult</subject><subject>Aged</subject><subject>Aged, 80 and over</subject><subject>Allergens - immunology</subject><subject>Allergies</subject><subject>anaphylaxis</subject><subject>Anaphylaxis - complications</subject><subject>Anaphylaxis - diagnosis</subject><subject>Anaphylaxis - epidemiology</subject><subject>Anaphylaxis - etiology</subject><subject>atopic disease</subject><subject>Comorbidity</subject><subject>Female</subject><subject>Humans</subject><subject>Hymenoptera</subject><subject>hymenoptera venom</subject><subject>IgE sensitization</subject><subject>Immunoglobulin E - immunology</subject><subject>Male</subject><subject>Mast Cells - immunology</subject><subject>Mast Cells - metabolism</subject><subject>mastocytosis</subject><subject>Mastocytosis, Systemic - complications</subject><subject>Medical research</subject><subject>Medicin och hälsovetenskap</subject><subject>Middle Aged</subject><subject>Prevalence</subject><subject>Risk Factors</subject><subject>Skin Tests</subject><subject>tryptase</subject><subject>Young Adult</subject><issn>0954-7894</issn><issn>1365-2222</issn><issn>1365-2222</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2014</creationdate><recordtype>article</recordtype><recordid>eNqNklmLFDEUhQtRnHb0wT8gAV8UpmaSVJbKY9OziaMiuDyGVOp2d2Zqm6TK7vr3pu1FEBrMS0L4zknuvSdJXhN8TuK6sGDOCaWUP0kmJBM8jWf6NJlgxVkqc8VOkhch3GOMM67y58kJZUQwyukkKW7dYok6D79MBY0F1M6RaUy3HCuzdgG5BnWmd9D0Aa1cv0RhDD3UzqLahL61Y9-GiKXIoOCaRQWpjawHBOsOvNtYvkyezU0V4NVuP02-X199m92md19uPsymd6nlTPE0UyrnnIIVCjIlZT4X3NLC5DKThBW0LDEosAYXYGRJCrAlZ6RkCpeFldJkp0m69Q0r6IZCd97Vxo-6NU7vrh7iCTSnAlMWeXWU73xb_hXthYRKlcmck6g9O6q9dD-muvULPQyaSpyzPOLvtnj0fRwg9Lp2wUJVmQbaIWjCFBWcKon_B8WxORnjEX37D3rfDr6JPdYk1hjflmJT5vstZX0bgof54bME6018dIyP_hOfyL7ZOQ5FDeWB3OclAhdbYOUqGI876dnVdG-5m4qLuVkfFMY_aBEny_XPzzdaXopciK-f9MfsNzUo3sE</recordid><startdate>201401</startdate><enddate>201401</enddate><creator>Gülen, T.</creator><creator>Hägglund, H.</creator><creator>Dahlén, B.</creator><creator>Nilsson, G.</creator><general>Blackwell Publishing Ltd</general><general>Wiley Subscription Services, Inc</general><scope>BSCLL</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7T5</scope><scope>H94</scope><scope>K9.</scope><scope>7X8</scope><scope>ADTPV</scope><scope>AOWAS</scope><scope>DF2</scope></search><sort><creationdate>201401</creationdate><title>High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience</title><author>Gülen, T. ; Hägglund, H. ; Dahlén, B. ; Nilsson, G.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c5495-3998552ec69e39778f65c2ba873714b2dd0e9eca0bea7d1becd541d490dbc77a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2014</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Aged, 80 and over</topic><topic>Allergens - immunology</topic><topic>Allergies</topic><topic>anaphylaxis</topic><topic>Anaphylaxis - complications</topic><topic>Anaphylaxis - diagnosis</topic><topic>Anaphylaxis - epidemiology</topic><topic>Anaphylaxis - etiology</topic><topic>atopic disease</topic><topic>Comorbidity</topic><topic>Female</topic><topic>Humans</topic><topic>Hymenoptera</topic><topic>hymenoptera venom</topic><topic>IgE sensitization</topic><topic>Immunoglobulin E - immunology</topic><topic>Male</topic><topic>Mast Cells - immunology</topic><topic>Mast Cells - metabolism</topic><topic>mastocytosis</topic><topic>Mastocytosis, Systemic - complications</topic><topic>Medical research</topic><topic>Medicin och hälsovetenskap</topic><topic>Middle Aged</topic><topic>Prevalence</topic><topic>Risk Factors</topic><topic>Skin Tests</topic><topic>tryptase</topic><topic>Young Adult</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Gülen, T.</creatorcontrib><creatorcontrib>Hägglund, H.</creatorcontrib><creatorcontrib>Dahlén, B.</creatorcontrib><creatorcontrib>Nilsson, G.</creatorcontrib><collection>Istex</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Immunology Abstracts</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>ProQuest Health &amp; Medical Complete (Alumni)</collection><collection>MEDLINE - Academic</collection><collection>SwePub</collection><collection>SwePub Articles</collection><collection>SWEPUB Uppsala universitet</collection><jtitle>Clinical and experimental allergy</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Gülen, T.</au><au>Hägglund, H.</au><au>Dahlén, B.</au><au>Nilsson, G.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience</atitle><jtitle>Clinical and experimental allergy</jtitle><addtitle>Clin Exp Allergy</addtitle><date>2014-01</date><risdate>2014</risdate><volume>44</volume><issue>1</issue><spage>121</spage><epage>129</epage><pages>121-129</pages><issn>0954-7894</issn><issn>1365-2222</issn><eissn>1365-2222</eissn><abstract>Summary Background Systemic mastocytosis (SM) is a clonal mast cells disorder characterized by the proliferation, accumulation and activation of mast cells in extracutaneous tissues. The clinical picture is heterogeneous and may range from asymptomatic to potentially fatal anaphylactic reactions due to excessive mast cell mediator release. Objective The aim of this study was to investigate the prevalence and trigger factors of anaphylactic reactions among adult SM patients. We also explored the clinical spectrum of mast cell mediator‐related symptoms in patients with SM. Methods This descriptive study was performed among 84 consecutive adult (≥ 18 years) patients those were diagnosed with SM according to WHO criteria. Sixty‐six of the patients also underwent a comprehensive allergy work‐up. Results Sixty of 84 patients with SM (71%) had bone marrow mast cell aggregates and fulfilled the major criteria for SM and 76 patients (91%) had indolent disease. Simultaneous occurrence of cutaneous mastocytosis was observed in 59 patients (70%). Thirty‐six patients (43%) had had at least one episode of an anaphylactic reaction. The clinical courses of the reactions were usually severe and patients often presented with syncope attacks (72%). Most patients reacted after hymenoptera venom stings (19/36; 53%). In 39% (14/36), a clear aetiology could not be determined. While males and females were equally frequent among the patients with SM, anaphylaxis patients showed a male predominance (61%). Anaphylactic reactions occurred more frequently in patients without cutaneous engagement. The rate of allergy sensitization was significantly higher in SM patients with anaphylaxis as compared with non‐anaphylaxis SM patients, 70% vs. 23%, respectively (P = 0.0002). Conclusions and Clinical Relevance Anaphylaxis is more prevalent in patients with SM, predominantly in patients with atopic SM. Hymenoptera venom‐induced and idiopathic anaphylaxis were the most frequent elicitors. Our findings implicate that all mastocytosis patients with anaphylaxis should undergo detailed allergological assessment before considering treatment and preventive measures.</abstract><cop>England</cop><pub>Blackwell Publishing Ltd</pub><pmid>24164252</pmid><doi>10.1111/cea.12225</doi><tpages>9</tpages></addata></record>
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subjects Adult
Aged
Aged, 80 and over
Allergens - immunology
Allergies
anaphylaxis
Anaphylaxis - complications
Anaphylaxis - diagnosis
Anaphylaxis - epidemiology
Anaphylaxis - etiology
atopic disease
Comorbidity
Female
Humans
Hymenoptera
hymenoptera venom
IgE sensitization
Immunoglobulin E - immunology
Male
Mast Cells - immunology
Mast Cells - metabolism
mastocytosis
Mastocytosis, Systemic - complications
Medical research
Medicin och hälsovetenskap
Middle Aged
Prevalence
Risk Factors
Skin Tests
tryptase
Young Adult
title High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience
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