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The problem of anaphylaxis and mastocytosis

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Abstract

Mastocytosis is a rare disease characterized by an elevated whole body mast cell number. Anaphylaxis is a severe, generalized hypersensitivity reaction with rapid onset. The problem of anaphylaxis and mastocytosis is due to strongly increased mediator release from the elevated mast cell number during allergic reactions. This explains the much higher prevalence of anaphylaxis in mastocytosis than in the general population and its severe and sometimes fatal course. Because of the increased risk of anaphylaxis in mastocytosis, all patients with severe or recurrent anaphylaxis should be analyzed for underlying mastocytosis by estimation of baseline serum tryptase. If this is elevated, patients also should be tested via skin examination for cutaneous mastocytosis and with a bone marrow biopsy. All patients with mastocytosis and anaphylaxis must be instructed about avoiding the responsible elicitors and should carry an emergency kit with adrenaline for self-application. In mastocytosis patients with anaphylaxis due to Hymenoptera stings, venom immunotherapy is recommended for life.

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References and Recommended Reading

  1. Reimers A, Müller U: Fatal outcome of a Vespula sting in a patient with mastocytosis after specific immunotherapy with honey bee venom. Swiss Med Wkly 2005, 135(Suppl 144):S14.

    Google Scholar 

  2. Müller U, Morris T, Bischof M, et al.: Combined active and passive immunotherapy in honeybee-sting allergy. J Allergy Clin Immunol 1986, 78:115–122.

    Article  PubMed  Google Scholar 

  3. Johansson SGO, Bieber TH, Dahl R, et al.: Revised nomenclature for allergy for global use: report of the Nomenclature Review Committee of the World Allergy Organization, October 2003. J Allergy Clin Immunol 2004, 113:832–836.

    Article  PubMed  CAS  Google Scholar 

  4. Sampson HA, Munoz-Furlong A, Campbell RL, et al.: Second symposium on the definition and management of anaphylaxis: summary report—Second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network symposium. J Allergy Clin Immunol 2006, 117:391–397.

    Article  PubMed  Google Scholar 

  5. Pumphrey RSH: Lessons for management of anaphylaxis from a study of fatal reactions. Clin Exp Allergy 2000, 30:1144–1150.

    Article  PubMed  CAS  Google Scholar 

  6. Yocum MW, Butterfield JH, Klein JS, et al.: Epidemiology of anaphylaxis in Olmstead County: a population based study. J Allergy Clin Immunol 1999, 104:452–456.

    Article  PubMed  CAS  Google Scholar 

  7. Helbling A, Thomas H, Müller U, Pichler WJ: Incidence of anaphylaxis with circulatory symptoms. A study over a 3-year period comprising 940 000 inhabitants of the Swiss Canton Bern. Clin Exp Allergy 2004, 34:285–290.

    Article  PubMed  CAS  Google Scholar 

  8. Bohlke K, Davis RL, DeStefano R, et al.: Epidemiology of anaphylaxis among children and adults enrolled in a health maintenance organization. J Allergy Clin Immunol 2004, 113:536–542.

    Article  PubMed  Google Scholar 

  9. Bresser H, Sandner CH, Rakoski J: Emergencies by insect stings in Munich 1992. Allergo J 2005, 7:373–376.

    Google Scholar 

  10. Peng MM, Jick H: A population based study of the incidence, cause and severity of anaphylaxis in the United Kingdom. Arch Intern Med 2004, 164:317–319.

    Article  PubMed  Google Scholar 

  11. Monneret-Vautrin DA, Morisset M, Flabee J, et al.: Epidemiology of life-threatening and lethal anaphylaxis: a review. Allergy 2005, 60:443–451.

    Article  Google Scholar 

  12. Valent P, Akin C, Escribano L, et al.: Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria. Eur J Clin Invest 2007, 37:435–453.

    Article  PubMed  CAS  Google Scholar 

  13. Castells MC: Mastocytosis: classification, diagnosis, and clinical presentation. Allergy Asthma Proc 2004, 25:33–36.

    PubMed  Google Scholar 

  14. Brockow K: Markers of severity for systemic mastocytosis [in German]. Allergo J 2006, 15:273–279.

    Google Scholar 

  15. Greenhawt M, Akin C: Mastocytosis and allergy. Curr Opin Allergy Clin Immunol 2007, 74:387–392.

    Google Scholar 

  16. Tefferi A, Pardanani A: Systemic mastocytosis: current concepts and treatment advances. Curr Hematol Rep 2004, 3:197–202.

    PubMed  Google Scholar 

  17. Valent P, Horny HP, Escribano L, et al.: Diagnostic criteria and classification of mastocytosis: a consensus proposal. Leuk Res 2001, 25:603–625.

    Article  PubMed  CAS  Google Scholar 

  18. Wolff K, Komar M, Petzelbauer P: Clinical and histopathological aspects of cutaneous mastocytosis. Leuk Res 2001, 25:519–528.

    Article  PubMed  CAS  Google Scholar 

  19. Hartmann K, Metcalfe DD: Pediatric mastocytosis. Hematol Oncol Clin North Am 2000, 14:625–640.

    Article  PubMed  CAS  Google Scholar 

  20. Akin C, Metcalfe DD: Systemic mastocytosis. Annu Rev Med 2004, 55:419–432.

    Article  PubMed  CAS  Google Scholar 

  21. Müller U, Helbling A, Hunziker T, et al.: Mastocytosis and atopy: a study in 33 patients with urticaria pigmentosa. Allergy 1990, 45:597–603.

    Article  PubMed  Google Scholar 

  22. González de Olano D, de la Hoz Caballer B, Núñez López R, et al.: Prevalence of allergy and anaphylactic symptoms in 219 adult and pediatric patients with mastocytosis in Spain: a study of the Spanish network on mastocytosis (REMA). Clin Exp Allergy 2007, 37:1547–1555.

    PubMed  Google Scholar 

  23. Brockow K, Jofer C, Behrendt H, Ring J: Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy 2008, 63:226–232.

    PubMed  CAS  Google Scholar 

  24. Müller U, Horat W, Wüthrich B, et al.: Anaphylaxis after Hymenoptera stings in 3 patients with urticaria pigmentosa. J Allergy Clin Immunol 1983, 72:685–689.

    Article  PubMed  Google Scholar 

  25. Price LA, Satko M: Bee venom allergy in a patient with urticaria pigmentosa. J Allergy Clin Immunol 1987, 79:407–409.

    Article  PubMed  CAS  Google Scholar 

  26. Kors JW, van Doormaal JJ, de Monchy JGR: Anaphylactoid shock following Hymenoptera sting as a presenting symptom of systemic mastocytosis. J Intern Med 1993, 233:255–258.

    Article  PubMed  CAS  Google Scholar 

  27. Fricker M, Helbling A, Schwartz LB, Müller U: Hymenoptera sting anaphylaxis and urticaria pigmentosa: clinical findings and results of venom immunotherapy in 10 patients. J Allergy Clin Immunol 1997, 100:11–15.

    Article  PubMed  CAS  Google Scholar 

  28. Oude Elberink JNG, de Monchy JGR, Kors JW, et al.: Fatal anaphylaxis after a yellow jacket sting in two patients with mastocytosis. J Allergy Clin Immunol 1997, 99:153–154.

    PubMed  CAS  Google Scholar 

  29. Wagner N, Fritze D, Przybilla B, et al.: Fatal anaphylactic sting reaction in a patient with mastocytosis. Int Arch Allergy Immunol 2008, 146:162–163.

    Article  PubMed  CAS  Google Scholar 

  30. Pumphrey RSH, Roberts ISD: Postmortem findings after fatal anaphylactic reactions. J Clin Pathol 2000, 53:273–276.

    Article  PubMed  CAS  Google Scholar 

  31. Dodd NJ, Bond MG: Fatal anaphylaxis in systemic mastocytosis. Clin Pathol 1979, 32:31–34.

    Article  CAS  Google Scholar 

  32. Vaughan ST, Jones GN: Systemic mastocytosis presenting as profound cardiovascular collapse during anesthesia. Anaesthesia 1998, 53:804–807.

    Article  PubMed  CAS  Google Scholar 

  33. Florian S, Kraut MT, Simonitch-Klupp I, et al.: Indolent systemic mastocytosis with elevated serum tryptase, absence of skin lesions, and recurrent severe anaphylactoid episodes. Int Arch Allergy Immunol 2005, 136:273–280.

    Article  PubMed  Google Scholar 

  34. Ruëff F, Placzek M, Przybilla B: Mastocytosis and Hymenoptera venom allergy. Curr Opin Allergy Clin Immunol 2006, 6:284–288.

    Article  PubMed  Google Scholar 

  35. Schwartz LB, Yunginger JW, Miller J, et al.: Time course of appearance and disappearance of human mast cell tryptase in the circulation after anaphylaxis. J Clin Invest 1989, 83:1551–1555.

    Article  PubMed  CAS  Google Scholar 

  36. Schwartz LB, Bradford TR, Rouse C, et al.: Development of a new, more sensitive immunoassay for human tryptase: use in systemic anaphylaxis. J Clin Immunol 1994, 14:190–204.

    Article  PubMed  CAS  Google Scholar 

  37. Ludolph-Hauser D, Ruëff F, Fries C, et al.: Constitutively raised serum concentrations of mast-cell tryptase and severe anaphylactic reactions to Hymenoptera stings. Lancet 2001, 357:361–362.

    Article  PubMed  CAS  Google Scholar 

  38. Haeberli G, Brönnimann M, Hunziker T, et al.: Elevated basal serum tryptase and hymenoptera venom allergy: relation to severity of sting reactions and to safety and efficacy of venom immunotherapy. Clin Exp Allergy 2003, 33:1216–1220.

    Article  PubMed  CAS  Google Scholar 

  39. Kucharewicz I, Bodzenta-Lukaszyk A, Szymanski W, et al.: Basal serum tryptase level correlates with severity of Hymen optera sting and age. J Investig Allergol Clin Immunol 2007, 17:65–69.

    PubMed  CAS  Google Scholar 

  40. Ruëff F, Bauer M, Placzek M, et al.: Treatment of mastocytosis. Allergologie 2006, 29:362–369.

    Google Scholar 

  41. Escribano L, Akin C, Castells M, Schwartz LB.: Current options in the treatment of mast cell mediator-related symptoms of mastocytosis. Inflamm Allergy Drug Targets 2006, 5:61–67.

    Article  PubMed  CAS  Google Scholar 

  42. Ring J, Brockow K, Duda D, et al.: Emergency treatment of anaphylactic reactions. Allergo J 2007, 16:420–434.

    Google Scholar 

  43. Wolff K: Treatment of cutaneous mastocytosis. Int Arch Allergy Immunol 2002, 127:156–159.

    Article  PubMed  CAS  Google Scholar 

  44. González de Olano D, Alvarez-Twose I, Esteban-López MI, et al.: Safety and effectiveness of immunotherapy in patients with indolent systemic mastocytosis presenting with Hymenoptera venom anaphylaxis. J Allergy Clin Immunol 2008, 121:519–526.

    Article  PubMed  Google Scholar 

  45. Bonadonna P, Zanotti R, Caruso B, et al.: Allergen specific immunotherapy is safe and effective in patients with mastocytosis and Hymenoptera allergy. J Allergy Clin Immunol 2008, 121:256–257.

    Article  PubMed  CAS  Google Scholar 

  46. Kontou-Fili K: High omalizumab dose controls recurrent reactions to venom immunotherapy in indolent systemic mastocytosis. Allergy 2008, 63:376–378.

    Article  PubMed  CAS  Google Scholar 

  47. Reimers A, Hari Y, Müller U: Reduction of side effects from ultra-rush immunotherapy with honey bee venom by pre-treatment with fexofenadine: a double-blind placebo-controlled trial. Allergy 2000, 55:484–488.

    Article  PubMed  CAS  Google Scholar 

  48. Bonifazi F, Jutel M, Bilo BM, et al.: Prevention and treatment of Hymenoptera venom allergy. Allergy 2005, 60:1459–1470.

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Allergy Division, Department of Medicine, Spital Ziegler, Spitalnetzbern, Morillonstrasse 75-91, CH-3007, Bern, Switzerland

    Ulrich R. Müller

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  1. Ulrich R. Müller
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  2. Gabrielle Haeberli
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Correspondence to Ulrich R. Müller.

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Müller, U.R., Haeberli, G. The problem of anaphylaxis and mastocytosis. Curr Allergy Asthma Rep 9, 64–70 (2009). https://doi.org/10.1007/s11882-009-0010-9

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