Roberta Zanotti1, Carla Lombardo2, Giovanni Passalacqua3, Cristian Caimmi4, Massimiliano Bonifacio1, Giovanna De Matteis5, Omar Perbellini6, Maurizio Rossini7, Donatella Schena8, Moira Busa9, Maria Cinzia Marcotulli10, Maria Beatrice Bilò11, Maurizio Franchini12, Giovanni Marchi13, Livio Simioni14, Patrizia Bonadonna15. 1. Department of Medicine, Section of Hematology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Multidisciplinary Mastocytosis Outpatient Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 2. Allergy Unit, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 3. Allergy and Respiratory Diseases, IRCCS San Martino-IST, University of Genoa, Genoa, Italy. Electronic address: passalacqua@unige.it. 4. Department of Medicine, Section of Rheumatology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 5. Clinical Chemistry and Haematology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 6. Department of Medicine, Section of Hematology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 7. Multidisciplinary Mastocytosis Outpatient Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Department of Medicine, Section of Rheumatology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 8. Multidisciplinary Mastocytosis Outpatient Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Department of Medicine, Section of Dermatology, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy. 9. Dermatology Unit, ULSS 13, Mirano, Venice, Italy. 10. Pulmonology Unit, Azienda Ospedaliera di Desenzano, Brescia, Italy. 11. Allergy Unit, Department of Internal Medicine, Ospedali Riuniti di Ancona, Ancona, Italy. 12. Allergy Service, ULSS 10, Jesolo, Venice, Italy. 13. Allergy Service, ULSS 21 Legnago, Verona, Italy. 14. Department of Medicine, Allergy Service, ULSS 2 di Feltre, Belluno, Italy. 15. Multidisciplinary Mastocytosis Outpatient Clinic, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy; Allergy Unit, Azienda Ospedaliera Universitaria Integrata of Verona, Verona, Italy.
Abstract
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.
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.
Authors: M Rossini; R Zanotti; G Orsolini; G Tripi; O Viapiana; L Idolazzi; A Zamò; P Bonadonna; V Kunnathully; S Adami; D Gatti Journal: Osteoporos Int Date: 2016-02-18 Impact factor: 4.507
Authors: Peter Valent; Patrizia Bonadonna; Karin Hartmann; Sigurd Broesby-Olsen; Knut Brockow; Joseph H Butterfield; Massimo Triggiani; Jonathan J Lyons; Joanna N G Oude Elberink; Michel Arock; Dean D Metcalfe; Cem Akin Journal: Int Arch Allergy Immunol Date: 2019-06-28 Impact factor: 2.749
Authors: Sarah C Glover; Melody C Carter; Peter Korošec; Patrizia Bonadonna; Lawrence B Schwartz; Joshua D Milner; George H Caughey; Dean D Metcalfe; Jonathan J Lyons Journal: Ann Allergy Asthma Immunol Date: 2021-08-13 Impact factor: 6.248