Clonal mast cell disorders in patients with severe Hymenoptera venom allergy and normal serum tryptase levels

JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY

卷:136

Article

Zanotti, Roberta^1,2  Lombardo, Carla³  Passalacqua, Giovanni⁷  Caimmi, Cristian⁴  Bonifacio, Massimiliano^1,2  De Matteis, Giovanna⁵  Perbellini, Omar¹  Rossini, Maurizio^2,4  Schena, Donatella^2,6  Busa, Moira⁸  Marcotulli, Maria Cinzia⁹  Bilo, Maria Beatrice¹⁰  Franchini, Maurizio¹¹  Marchi, Giovanni¹²  Simioni, Livio¹³  Bonadonna, Patrizia^2,3 

[1] Azienda Osped Univ Integrata Verona, Dept Med, Sect Hematol, Verona, Italy

[2] Azienda Osped Univ Integrata Verona, Multidisciplinary Mastocytosis Outpatient Clin, Verona, Italy

[3] Azienda Osped Univ Integrata Verona, Allergy Unit, Verona, Italy

[4] Azienda Osped Univ Integrata Verona, Dept Med, Rheumatol Sect, Verona, Italy

[5] Azienda Osped Univ Integrata Verona, Clin Chem & Haematol, Verona, Italy

[6] Azienda Osped Univ Integrata Verona, Dept Med, Dermatol Sect, Verona, Italy

[7] Univ Genoa, IRCCS San Martino IST, Allergy & Resp Dis, I-16126 Genoa, Italy

[8] ULSS 13, Dermatol Unit, Venice, Italy

[9] Azienda Osped Desenzano, Pulmonol Unit, Brescia, Italy

[10] Osped Riuniti Ancona, Dept Internal Med, Allergy Unit, Verona, Italy

[11] ULSS 10, Allergy Serv, Venice, Italy

[12] ULSS 21 Legnago, Allergy Serv, Verona, Italy

[13] ULSS 2 Feltre, Allergy Serv, Dept Med, Belluno, Italy

关键词: Systemic mastocytosis;    anaphylaxis;    tryptase;    Hymenoptera venom allergy;   

DOI  :  10.1016/j.jaci.2014.11.035

来源: Elsevier

【 摘 要 】

Background: Systemic mastocytosis is a clonal mast cell (MC) disease that can lead to potentially fatal anaphylactic reactions caused by excessive MC mediator release. The prevalence of mastocytosis in patients with Hymenoptera venom allergy is high, and thus the disease should be suspected in patients with severe reactions caused by Hymenoptera stings and increased serum basal tryptase (SBT) levels. Objective: We sought to evaluate the presence of clonal MC disorders in patients seen at our mastocytosis center with Hymenoptera sting-induced anaphylaxis, documented hypotension, absence of urticaria pigmentosa, and normal SBT levels. Methods: Twenty-two patients with Hymenoptera sting-induced anaphylaxis, without skin lesions, and with tryptase levels of less than 11.4 ng/mL underwent bone marrow evaluation. Bone mineral density was assessed in those patients with ascertained mastocytosis. Results: In 16 of 22 patients, a diagnosis of indolent mastocytosis could be established, and 1 patient had a monoclonal MC activation syndrome. Patients with mastocytosis had higher SBT levels (P = .03) but only rarely had angioedema/urticaria associated with hypotension (P = .004). Conclusions: The absence of urticaria or angioedema in severe reactions to Hymenoptera stings with hypotension might represent the most relevant factor in identifying patients with mastocytosis, regardless of their serum tryptase levels.

【 授权许可】

Free