Mast cell activation syndrome

Valent P, Akin C, Arock M, et al. Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal. Int Arch Allergy Immunol. 2012;157(3):215-25.Full text  Abstract

Weiler CR, Austen KF, Akin C, et al. AAAAI Mast Cell Disorders Committee Work Group report: Mast cell activation syndrome (MCAS) diagnosis and management. J Allergy Clin Immunol. 2019 Oct;144(4):883-96.Full text  Abstract

Gülen T, Akin C. Anaphylaxis and mast cell disorders. Immunol Allergy Clin North Am. 2022 Feb;42(1):45-63.Full text  Abstract

Gülen T, Akin C, Bonadonna P, et al. Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review. J Allergy Clin Immunol Pract. 2021 Nov;9(11):3918-28.Full text  Abstract

Lieberman P, Nicklas RA, Randolph C, et al. Anaphylaxis--a practice parameter update 2015. Ann Allergy Asthma Immunol. 2015;115:341-84. Abstract

Shaker MS, Wallace DV, Golden DBK, et al. Anaphylaxis-a 2020 practice parameter update, systematic review, and grading of recommendations, assessment, development and evaluation (GRADE) analysis. J Allergy Clin Immunol. 2020 Apr;145(4):1082-123.Full text  Abstract

1. Valent P, Akin C. Doctor, I think I am suffering from MCAS: Differential diagnosis and separating facts from fiction. J Allergy Clin Immunol Pract. 2019 Apr;7(4):1109-14.Full text  Abstract

2. Valent P, Akin C, Arock M, et al. Definitions, criteria and global classification of mast cell disorders with special reference to mast cell activation syndromes: a consensus proposal. Int Arch Allergy Immunol. 2012;157(3):215-25.Full text  Abstract

3. Weiler CR, Austen KF, Akin C, et al. AAAAI Mast Cell Disorders Committee Work Group report: Mast cell activation syndrome (MCAS) diagnosis and management. J Allergy Clin Immunol. 2019 Oct;144(4):883-96.Full text  Abstract

4. Gülen T, Akin C. Anaphylaxis and mast cell disorders. Immunol Allergy Clin North Am. 2022 Feb;42(1):45-63.Full text  Abstract

5. Valent P, Akin C, Bonadonna P, et al. Proposed diagnostic algorithm for patients with suspected mast cell activation syndrome. J Allergy Clin Immunol Pract. 2019 Apr;7(4):1125-33.e1.Full text  Abstract

6. Lieberman P, Camargo CA Jr, Bohlke K, et al. Epidemiology of anaphylaxis: findings of the American College of Allergy, Asthma and Immunology Epidemiology of Anaphylaxis Working Group. Ann Allergy Asthma Immunol. 2006;97:596-602. Abstract

7. NHS Digital. Hospital admissions for allergies and anaphylactic shock. Jul 2019 [internet publication].Full text

8. Gaspar A, Santos N, Faria E, et al. Anaphylaxis: a decade of a Nationwide Allergy Society Registry. J Investig Allergol Clin Immunol. 2021 Feb 21;32(1):23-32. Abstract

9. Chaaban MR, Stuart J, Watley D, et al. Recurrent anaphylaxis in the United States: time of onset and risk factors. Int Forum Allergy Rhinol. 2020 Mar;10(3):320-7. Abstract

10. Cohen SS, Skovbo S, Vestergaard H, et al. Epidemiology of systemic mastocytosis in Denmark. Br J Haematol. 2014 Aug;166(4):521-8.Full text  Abstract

11. van Doormaal JJ, Arends S, Brunekreeft KL, et al. Prevalence of indolent systemic mastocytosis in a Dutch region. J Allergy Clin Immunol. 2013 May;131(5):1429-31.e1. Abstract

12. Schuler CF 4th, Volertas S, Khokhar D, et al. Prevalence of mastocytosis and Hymenoptera venom allergy in the United States. J Allergy Clin Immunol. 2021 Nov;148(5):1316-23.Full text  Abstract

13. Afrin LB, Ackerley MB, Bluestein LS, et al. Diagnosis of mast cell activation syndrome: a global "consensus-2". Diagnosis (Berl). 2020;8(2):137-52.Full text  Abstract

14. Theoharides TC, Valent P, Akin C. Mast Cells, mastocytosis, and related disorders. N Engl J Med. 2015 Jul 9;373(2):163-72. Abstract

15. Castells MC, Irani AM, Schwartz LB. Evaluation of human peripheral blood leukocytes for mast cell tryptase. J Immunol. 1987 Apr 1;138(7):2184-9. Abstract

16. Jogie-Brahim S, Min HK, Fukuoka Y, et al. Expression of alpha-tryptase and beta-tryptase by human basophils. J Allergy Clin Immunol. 2004 Jun;113(6):1086-92.Full text  Abstract

17. Castells MC, Hornick JL, Akin C. Anaphylaxis after hymenoptera sting: is it venom allergy, a clonal disorder, or both? J Allergy Clin Immunol Pract. 2015 May-Jun;3(3):350-5. Abstract

18. Gülen T, Akin C, Bonadonna P, et al. Selecting the right criteria and proper classification to diagnose mast cell activation syndromes: a critical review. J Allergy Clin Immunol Pract. 2021 Nov;9(11):3918-28.Full text  Abstract

19. Gülen T, Hägglund H, Dahlén B, et al. High prevalence of anaphylaxis in patients with systemic mastocytosis - a single-centre experience. Clin Exp Allergy. 2014 Jan;44(1):121-9. Abstract

20. Gülen T, Ljung C, Nilsson G, et al. Risk factor analysis of anaphylactic reactions in patients with systemic mastocytosis. J Allergy Clin Immunol Pract. 2017 Sep-Oct;5(5):1248-55. Abstract

21. Brockow K, Jofer C, Behrendt H, et al. Anaphylaxis in patients with mastocytosis: a study on history, clinical features and risk factors in 120 patients. Allergy. 2008 Feb;63(2):226-32. Abstract

22. Gülen T, Hägglund H, Sander B, et al. The presence of mast cell clonality in patients with unexplained anaphylaxis. Clin Exp Allergy. 2014 Sep;44(9):1179-87. Abstract

23. Turner PJ, Gowland MH, Sharma V, et al. Increase in anaphylaxis-related hospitalizations but no increase in fatalities: an analysis of United Kingdom national anaphylaxis data, 1992-2012. J Allergy Clin Immunol. 2015 Apr;135(4):956-63.e1.Full text  Abstract

24. Jerschow E, Lin RY, Scaperotti MM, et al. Fatal anaphylaxis in the United States, 1999-2010: temporal patterns and demographic associations. J Allergy Clin Immunol. 2014 Dec;134(6):1318-28.e7.Full text  Abstract

25. Lyons JJ, Chovanec J, O'Connell MP, et al. Heritable risk for severe anaphylaxis associated with increased α-tryptase-encoding germline copy number at TPSAB1. J Allergy Clin Immunol. 2021 Feb;147(2):622-32.Full text  Abstract

26. Greiner G, Sprinzl B, Górska A, et al. Hereditary α tryptasemia is a valid genetic biomarker for severe mediator-related symptoms in mastocytosis. Blood. 2021 Jan 14;137(2):238-47.Full text  Abstract

27. Chollet MB, Akin C. Hereditary alpha tryptasemia is not associated with specific clinical phenotypes. J Allergy Clin Immunol. 2022 Feb;149(2):728-35.e2.Full text  Abstract

28. Valent P, Akin C, Hartmann K, et al. Updated diagnostic criteria and classification of mast cell disorders: a consensus proposal. Hemasphere. 2021 Nov;5(11):e646.Full text  Abstract

29. Akin C, Valent P, Metcalfe DD. Mast cell activation syndrome: Proposed diagnostic criteria. J Allergy Clin Immunol. 2010 Dec;126(6):1099-104.e4.Full text  Abstract

30. Valent P, Hartmann K, Bonadonna P, et al. Mast cell activation syndromes: Collegium Internationale Allergologicum update 2022. Int Arch Allergy Immunol. 2022;183(7):693-705.Full text  Abstract

31. Vanden Hoek TL, Morrison LJ, Shuster M, et al. Part 12: cardiac arrest in special situations: 2010 American Heart Association guidelines for cardiopulmonary resuscitation and emergency cardiovascular care. Circulation. 2010 Nov 2;122(18 suppl 3):S829-61.Full text  Abstract

32. Stojanovic S, Zubrinich C, Sverrild A, et al. Laryngoscopy diagnosis of inducible laryngeal obstruction during supervised challenge for suspected anaphylaxis. Clin Exp Allergy. 2022 Aug;52(8):924-8.Full text  Abstract

33. Bagos-Estevez AG, Ledford DK. Anaphylaxis: definition, epidemiology, diagnostic challenges, grading system. Immunol Allergy Clin North Am. 2022 Feb;42(1):1-11. Abstract

34. Motosue MS, Li JT, Campbell RL. Anaphylaxis: epidemiology and differential diagnosis. Immunol Allergy Clin North Am. 2022 Feb;42(1):13-25. Abstract

35. Lieberman P, Nicklas RA, Randolph C, et al. Anaphylaxis--a practice parameter update 2015. Ann Allergy Asthma Immunol. 2015;115:341-84. Abstract

36. Gülen T, Hägglund H, Dahlén B, et al. Mastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic disease. J Intern Med. 2016 Mar;279(3):211-28.Full text  Abstract

37. Brown SG. Clinical features and severity grading of anaphylaxis. J Allergy Clin Immunol. 2004 Aug;114(2):371-6.Full text  Abstract

38. Brown SG. Cardiovascular aspects of anaphylaxis: implications for treatment and diagnosis. Curr Opin Allergy Clin Immunol. 2005 Aug;5(4):359-64. Abstract

39. Hartmann K, Escribano L, Grattan C, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 2016 Jan;137(1):35-45.Full text  Abstract

40. Metcalfe DD. Regulation of normal and neoplastic human mast cell development in mastocytosis. Trans Am Clin Climatol Assoc. 2005;116:185-204.Full text  Abstract

41. Arber DA, Orazi A, Hasserjian R, et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia. Blood. 2016 May 19;127(20):2391-405.Full text  Abstract

42. Butterfield JH. Nontryptase urinary and hematologic biomarkers of mast cell expansion and mast cell activation: status 2022. J Allergy Clin Immunol Pract. 2022 Aug;10(8):1974-84. Abstract

43. Ravi A, Butterfield J, Weiler CR. Mast cell activation syndrome: improved identification by combined determinations of serum tryptase and 24-hour urine 11β-prostaglandin2α. J Allergy Clin Immunol Pract. 2014;2:775-778. Abstract

44. Divekar R, Butterfield J. Urinary 11β-PGF2α and N-methyl histamine correlate with bone marrow biopsy findings in mast cell disorders. Allergy. 2015 Oct;70(10):1230-8. Abstract

45. Butterfield J, Weiler CR. The utility of measuring urinary metabolites of mast cell mediators in systemic mastocytosis and mast cell activation syndrome. J Allergy Clin Immunol Pract. 2020 Sep;8(8):2533-41. Abstract

46. Gülen T, Möller Westerberg C, Lyberg K, et al. Assessment of in vivo mast cell reactivity in patients with systemic mastocytosis. Clin Exp Allergy. 2017 Jul;47(7):909-17. Abstract

47. Kristensen T, Vestergaard H, Bindslev-Jensen C, et al. Sensitive KIT D816V mutation analysis of blood as a diagnostic test in mastocytosis. Am J Hematol. 2014 May;89(5):493-8.Full text  Abstract

48. Valent P, Aberer E, Beham-Schmid C, et al. Guidelines and diagnostic algorithm for patients with suspected systemic mastocytosis: a proposal of the Austrian competence network (AUCNM). Am J Blood Res. 2013;3(2):174-80.Full text  Abstract

49. Ertugrul A, Bostanci I, Ozturk Kaymak A, et al. Pediatric cutaneous mastocytosis and c-KIT mutation screening. Allergy Asthma Proc. 2019 Mar 1;40(2):123-8. Abstract

50. Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017 Mar 16;129(11):1420-7.Full text  Abstract

51. Afrin LB, Butterfield JH, Raithel M, et al. Often seen, rarely recognized: mast cell activation disease--a guide to diagnosis and therapeutic options. Ann Med. 2016;48(3):190-201.Full text  Abstract

52. Afrin LB, Self S, Menk J, et al. Characterization of mast cell activation syndrome. Am J Med Sci. 2017;353:207-215. Abstract

53. Afrin LB. Mast cell activation disease and the modern epidemic of chronic inflammatory disease. Transl Res. 2016 Aug;174:33-59. Abstract

54. Lyons JJ, Sun G, Stone KD, et al. Mendelian inheritance of elevated serum tryptase associated with atopy and connective tissue abnormalities. J Allergy Clin Immunol. 2014 May;133(5):1471-4.Full text  Abstract

55. Lyons JJ, Yu X, Hughes JD, et al. Elevated basal serum tryptase identifies a multisystem disorder associated with increased TPSAB1 copy number. Nat Genet. 2016 Dec;48(12):1564-9.Full text  Abstract

56. Robey RC, Wilcock A, Bonin H, et al. Hereditary alpha-tryptasemia: UK prevalence and variability in disease expression. J Allergy Clin Immunol Pract. 2020 Nov-Dec;8(10):3549-56. Abstract

57. Vanden Hoek TL, Morrison LJ, Shuster M, et al. Part 12: cardiac arrest in special situations: 2010 American Heart Association Guidelines for Cardiopulmonary Resuscitation and Emergency Cardiovascular Care. Circulation. 2010 Nov 2;122(suppl 3):S829-61.Full text  Abstract

58. Schwartz LB. Diagnostic value of tryptase in anaphylaxis and mastocytosis. Immunol Allergy Clin North Am. 2006 Aug;26(3):451-63. Abstract

59. Choy AC, Patterson R, Patterson DR, et al. Undifferentiated somatoform idiopathic anaphylaxis: nonorganic symptoms mimicking idiopathic anaphylaxis. J Allergy Clin Immunol. 1995 Dec;96(6 pt 1):893-900.Full text  Abstract

60. Roberts LJ 2nd. Carcinoid syndrome and disorders of systemic mast-cell activation including systemic mastocytosis. Endocrinol Metab Clin North Am. 1988 Jun;17(2):415-36. Abstract

61. Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65. Abstract

62. Shaker MS, Wallace DV, Golden DBK, et al. Anaphylaxis-a 2020 practice parameter update, systematic review, and grading of recommendations, assessment, development and evaluation (GRADE) analysis. J Allergy Clin Immunol. 2020 Apr;145(4):1082-123.Full text  Abstract

63. Muraro A, Worm M, Alviani C, et al. EAACI guidelines: Anaphylaxis (2021 update). Allergy. 2022 Feb;77(2):357-77.Full text  Abstract

64. Lieberman PL. Recognition and first-line treatment of anaphylaxis. Am J Med. 2014 Jan;127(suppl 1):S6-11. Abstract

65. Perkins GD, Graesner JT, Semeraro F, et al. European Resuscitation Council guidelines 2021: executive summary. Resuscitation. 2021 Apr;161:1-60.Full text  Abstract

66. Roberts LJ 2nd, Turk JW, Oates JA. Shock syndrome associated with mastocytosis: pharmacologic reversal of the acute episode and therapeutic prevention of recurrent attacks. Adv Shock Res. 1982;8:145-52. Abstract

67. Turk J, Oates JA, Roberts LJ 2nd. Intervention with epinephrine in hypotension associated with mastocytosis. J Allergy Clin Immunol. 1983 Feb;71(2):189-92.Full text  Abstract

68. Resuscitation Council (UK). Emergency treatment of anaphylactic reactions: guidelines for healthcare providers. May 2021 [internet publication].Full text

69. Campbell DE. Anaphylaxis management: time to re-evaluate the role of corticosteroids. J Allergy Clin Immunol Pract. 2019 Sep-Oct;7(7):2239-40.Full text  Abstract

70. Gülen T, Akin C. Pharmacotherapy of mast cell disorders. Curr Opin Allergy Clin Immunol. 2017 Aug;17(4):295-303. Abstract

71. Jarkvist J, Salehi C, Akin C, et al. Venom immunotherapy in patients with clonal mast cell disorders: IgG4 correlates with protection. Allergy. 2020 Jan;75(1):169-77. Abstract

72. Escribano L, Akin C, Castells M, et al. Current options in the treatment of mast cell mediator-related symptoms in mastocytosis. Inflamm Allergy Drug Targets. 2006 Jan;5(1):61-77. Abstract

73. Cardet JC, Akin C, Lee MJ. Mastocytosis: update on pharmacotherapy and future directions. Expert Opin Pharmacother. 2013 Oct;14(15):2033-45.Full text  Abstract

74. Siebenhaar F, Akin C, Bindslev-Jensen C, et al. Treatment strategies in mastocytosis. Immunol Allergy Clin North Am. 2014 May;34(2):433-47.Full text  Abstract

75. Czarnetzki BM. A double-blind cross-over study of the effect of ketotifen in urticaria pigmentosa. Dermatologica. 1983;166(1):44-7. Abstract

76. Nurmatov UB, Rhatigan E, Simons FE, et al. H1-antihistamines for primary mast cell activation syndromes: a systematic review. Allergy. 2015 Sep;70(9):1052-61.Full text  Abstract

77. Tolar J, Tope WD, Neglia JP. Leukotriene-receptor inhibition for the treatment of systemic mastocytosis. N Engl J Med. 2004 Feb 12;350(7):735-6. Abstract

78. Turner PJ, Kemp AS, Rogers M, et al. Refractory symptoms successfully treated with leukotriene inhibition in a child with systemic mastocytosis. Pediatr Dermatol. 2012 Mar-Apr;29(2):222-3. Abstract

79. Medicines and Healthcare products Regulatory Agency. Montelukast (Singulair): reminder of the risk of neuropsychiatric reactions Sep 2019 [internet publication].Full text

80. European Medicines Agency. Montelukast: CMDh scientific conclusions and grounds for the variation to the terms of the marketing authorisations. 2019 [internet publication].Full text

81. US Food and Drug Admisitration. FDA requires boxed warning about serious mental health side effects for asthma and allergy drug montelukast (Singulair); advises restricting use for allergic rhinitis. Mar 2020 [internet publication].Full text

82. Ting S. Ketotifen and systemic mastocytosis. J Allergy Clin Immunol. 1990 Apr;85(4):818.Full text  Abstract

83. Carter MC, Robyn JA, Bressler PB, et al. Omalizumab for the treatment of unprovoked anaphylaxis in patients with systemic mastocytosis. J Allergy Clin Immunol. 2007 Jun;119(6):1550-1.Full text  Abstract

84. Broesby-Olsen S, Vestergaard H, Mortz CG, et al. Omalizumab prevents anaphylaxis and improves symptoms in systemic mastocytosis: Efficacy and safety observations. Allergy. 2018 Jan;73(1):230-8. Abstract

85. Carter MC, Maric I, Brittain EH, et al. A randomized double-blind, placebo-controlled study of omalizumab for idiopathic anaphylaxis. J Allergy Clin Immunol. 2021 Mar;147(3):1004-10.e2.Full text  Abstract

86. Distler M, Maul JT, Steiner UC, et al. Efficacy of omalizumab in mastocytosis: allusive indication obtained from a prospective, double-blind, multicenter study (XOLMA Study). Dermatology. 2020;236(6):529-39. Abstract

87. Akin C, Arock M, Valent P. Tyrosine kinase inhibitors for the treatment of indolent systemic mastocytosis: Are we there yet? J Allergy Clin Immunol. 2022 Jun;149(6):1912-8. Abstract

88. Gotlib J, Kluin-Nelemans HC, George TI, et al. Efficacy and safety of midostaurin in advanced systemic mastocytosis. N Engl J Med. 2016 Jun 30;374(26):2530-41.Full text  Abstract

89. Valent P, Akin C, Hartmann K, et al. Midostaurin: a magic bullet that blocks mast cell expansion and activation. Ann Oncol. 2017 Oct 1;28(10):2367-76.Full text  Abstract

90. Hartmann K, Gotlib J, Akin C, et al. Midostaurin improves quality of life and mediator-related symptoms in advanced systemic mastocytosis. J Allergy Clin Immunol. 2020 Aug;146(2):356-66.e4.Full text  Abstract

91. Akin C, Elberink HO, Gotlib J, et al. PIONEER: a randomized, double-blind, placebo-controlled, phase 2 study of avapritinib in patients with indolent or smoldering systemic mastocytosis (SM) with symptoms inadequately controlled by standard therapy. J Allergy Clin Immunol 2020;145(2):AB336.

92. Kudlaty E, Perez M, Stein BL, et al. Systemic mastocytosis with an associated hematologic neoplasm complicated by recurrent anaphylaxis: Prompt resolution of anaphylaxis with the addition of avapritinib. J Allergy Clin Immunol Pract. 2021 Jun;9(6):2534-6. Abstract

93. Kluin-Nelemans HC, Jansen JH, Breukelman H, et al. Response to interferon alfa-2b in a patient with systemic mastocytosis. N Engl J Med. 1992 Feb 27;326(9):619-23.Full text  Abstract

94. Wimazal F, Geissler P, Shnawa P, et al. Severe life-threatening or disabling anaphylaxis in patients with systemic mastocytosis: a single-center experience. Int Arch Allergy Immunol. 2012;157(4):399-405. Abstract