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maintenance of certifi cation-cme review mast cell activation syndromes min jung lee, md; and cem akin, md, phd department of medicine, brigham and womens hospital, division of rheumatology, immunology, and allergy, boston, massachusetts a r t i c l ei n f o article history: received for publication september 4, 2012. received in revised form february 7, 2013. accepted for publication february 10, 2013. instructions credit can now be obtained, free for a limited time, by reading the review article in this issue and completing all activity components. please note the instructions listed below: ! review the target audience, learning objectives and all disclosures. ! complete the pre-test online at (click on the cme heading). ! follow the online instructions to read the full version of the article, including the clinical vignette; refl ect on all content as to how it may be applicable to your practice. ! complete the post-test/evaluation and claim credit earned; at this time, you will have earned up to 1.0 ama pra category 1 credittm. please note that the minimum passing score on the post-test is 70%. ! approximately 4-6 weeks later you will receive an online assessment regarding your application of this article to your practice. once you have completed this assessment, you will be eligible to receive moc part ii credit from the american board of allergy and immunology. release date: july 1, 2013 expiration date: june 30, 2015 estimated time to complete: 60 minutes target audience: physicians involved in providing patient care in the fi eld of allergy/asthma/immunology learning objectives: at the conclusion of this activity, participants should be able to: ! describe the classifi cation of mast cell activation disorders with emphasis on monoclonal mast cell activation syndrome (mmas) and idiopathic mast cell activation syndrome (mcas) ! discuss the clinical presentations, evaluation, diagnostic approach and management of mmas and mcas disorders accreditation: the american college of allergy, asthma e-mail: . contents lists available at sciverse sciencedirect 1081-1206/13/$36.00 - see front matter ! 2013 american college of allergy, asthma respiratory: broncho- spasm; cardiovascular: hypotension or syncope; and skin: fl ushing, itching and less commonly urticaria and angioedema) 2. increase in release of mast cell markers, such as tryptase, 24-hour urine histamine metabolites, or prostaglandin d2or its metabolite (11-b-prostaglandin f2) with symptoms 3. response in clinical symptoms to antimediator therapy athe proposed international consensus criteria for mast cell activation syndrome should meet all of these 3 criteria. m.j. lee and c. akin / ann allergy asthma immunol 111 (2013) 5e87 signifi cant elevation of their baseline tryptase levels during symptomaticperiods,hypotensiveanaphylacticepisodes,or anaphylactic hymenoptera hypersensitivity are candidates for more aggressive diagnostic approaches, such as bone marrow biopsy, to look for evidence of clonal mast cell disease, such as sm or mmas. references 1 valent p, akin c, escribano l, et al. standards and standardization in masto- cytosis: consensus statements on diagnostics, treatment recommendations and response criteria. eur j clin invest. 2007;37:435. 2 akin c, scott lm, kocabas cn, et al. demonstration of an aberrant mast-cell population with clonal markers in a subset of patients with “idiopathic” anaphylaxis. blood. 2007;110:2331. 3 swedlow sh, camp e, harris nl, et al, eds. who classifi cation of tumours of haematopoietic and lymphoid tissues. 4th ed. lyon, france: iarc; 2008. 4 bonadonna p, perbellini o, passalacqua g, et al. clonal mast cell disorders in patients with systemic reactions to hymenoptera stings and increased serum tryptase levels. j allergy clin immunol. 2009;123:680. 5 lim k, terfferi a, lasho t, et al. systemic mastocytosis in 342 consecutive adults: survival studies and prognostic factors. blood. 2009;113:5727e5736. 6 alvarez-twose i, gonzlez de olano d, snchez-muoz l, et al. clinical, bio- logical, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms. j allergy clin immunol. 2010; 125:1269. 7 akin c, valent p, metcalfe dd. mast cell activation syndrome: proposed diagnostic criteria. j allergy clin immunol. 2010;126:1099. 8 hamilton mj, hornick jl, akin c, et al. mast cell activation syndrome: a newly recognized disorder with systemic clinical manifestations. j allergy clin immunol. 2011;128:147. 9 molderings gj, brettner s, homann j, et al. mast cell activation disease: a conscise practical guide for diagnostic workup and therapeutic options. j hematol oncol. 2011;4:10. 10 valent p, akin c, arock m, et al. defi nitions, criteria and global classifi cation of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal. int arch allergy immunol. 2012;157:215. 11 worobec as. treatment of systemic mast cell disorders. hematol oncol clin north am. 2000;14:659. 12 dykewicz ms, wong ss, patterson r, harris ke. evaluation of ketotifen in corticosteroid-dependent idiopathic anaphylaxis. ann allergy. 1990;65:406. 13 soter na, austen kf, wasserman si. oral disodium cromoglycate in the treatment of systemic mastocytosis. n engl j med. 1979;301:465. 14 tolar j, tope wd, neglia jp. leukotriene-receptor inhibition for the treatment of systemic mastocytosis. n engl j med. 2004;350:735. 15 n-methylhistamine in urine; and 11-beta-prostaglandin f2 al
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