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خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : 3 مورد
نسخه الکترونیک
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Comparison of clinical and diagnostic features for systemic mastocytosis, mast cell activation syndromes, and idiopathic anaphylaxis

Comparison of clinical and diagnostic features for systemic mastocytosis, mast cell activation syndromes, and idiopathic anaphylaxis
  Systemic mastocytosis Monoclonal mast cell activation syndrome (MMAS) Idiopathic mast cell activation syndrome (IMCAS) Idiopathic anaphylaxis
Baseline tryptase* >20 Normal or mildly increased Normal or mildly increased Normal
kit D816V + + – –
Multifocal mast cell aggregates (each with ≥15 mast cells) in the bone marrow + – – –
Aberrant CD25 on bone marrow and other noncutaneous mast cells + + – –
Urticaria pigmentosa +/– – – –
Mediator-release symptoms + + + +
Hypotensive episodes +/– +/– +/– +/–
Urine N-MH or PGD2 Increased at baseline Increased during symptoms Increased during symptoms Increased during symptoms
Response to antimediator therapy + + + +/–
N-MH: N-methylhistamine; PGD2: prostaglandin D2.
* Elevations in serum tryptase corresponding to symptoms (particularly hypotension) may be seen in all four disorders. Increases in tryptase greater than 1.2 x baseline value + 2 ng/mL are considered significant. For example, if a patient's baseline total tryptase was 5 ng/mL, a value of 8 ng/mL would represent a significant increase.
¶ Urticaria pigmentosa has been renamed monomorphic maculopapular cutaneous mastocytosis.
Modified from: Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol 2010; 126:1099. Illustration used with the permission of Elsevier Inc. All rights reserved.
Graphic 74223 Version 16.0

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