Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms Iván Álvarez-Twose,

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Presentation transcript:

Clinical, biological, and molecular characteristics of clonal mast cell disorders presenting with systemic mast cell activation symptoms Iván Álvarez-Twose, MD, David González de Olano, MD, Laura Sánchez-Muñoz, MD, PhD, Almudena Matito, MD, Maria I. Esteban- López, MD, Arantza Vega, MD, PhD, Maria Belén Mateo, MD, Maria D. Alonso Díaz de Durana, MD, Belén de la Hoz, MD, PhD, Maria D. del Pozo Gil, MD, PhD, Teresa Caballero, MD, Ana Rosado, MD, Isabel Sánchez Matas, MD, Cristina Teodósio, MSc, María Jara- Acevedo, Msc, Manuela Mollejo, MD, PhD, Andrés García-Montero, PhD, Alberto Orfao, MD, PhD, Luis Escribano, MD, PhD Journal of Allergy and Clinical Immunology Volume 125, Issue 6, Pages 1269-1278.e2 (June 2010) DOI: 10.1016/j.jaci.2010.02.019 Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 1 Distribution of nc-MCAD and ISMs- patients (n = 80) according to the number of acute episodes after a median follow-up before the referral of 71 months (8-386 months; A) and the frequency of different triggers for the release of MC mediators (B) in ISMs- (n = 48) vs nc-MCAD (n = 32). No statistically significant differences regarding the median follow-up were found between clonal and nonclonal patients. In contrast, the median (range) follow-up of patients with a single MC mediators release episode was significantly lower (P < .001) than that of patients with >1 episode: 44 months (8-380 months) vs 103 months (13-386 months). Journal of Allergy and Clinical Immunology 2010 125, 1269-1278.e2DOI: (10.1016/j.jaci.2010.02.019) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 2 Overall distribution of clinical symptoms associated with the most severe MC mediators release episodes in ISMs- (n = 48) vs nc-MCAD patients (n = 32). Journal of Allergy and Clinical Immunology 2010 125, 1269-1278.e2DOI: (10.1016/j.jaci.2010.02.019) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Fig 3 Scoring model proposed as a screening tool for the presence of clonal MCs in patients presenting with anaphylaxis in the absence of skin mastocytosis before a BM study. Journal of Allergy and Clinical Immunology 2010 125, 1269-1278.e2DOI: (10.1016/j.jaci.2010.02.019) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

Distribution of clinical symptoms associated with the most severe MC mediators release episodes grouped by organ involvement in ISMs- (n = 48) vs. nc-MCAD patients (n = 32). Journal of Allergy and Clinical Immunology 2010 125, 1269-1278.e2DOI: (10.1016/j.jaci.2010.02.019) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions