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

42. 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

43. 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

44. 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

45. 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

46. 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

47. 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

48. American Academy of Allergy, Asthma and Immunology. Ten things physicians and patients should question.​ Choosing Wisely, an initiative of the ABIM Foundation. 2021 [internet publication].Full text

49. 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

50. 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

51. 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

52. 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

53. 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

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

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

56. 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

57. 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

58. 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

59. 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

60. 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

61. 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

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

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

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

67. 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

68. 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

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

70. 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

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

72. 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

73. 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

74. 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

75. 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

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

77. 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

78. 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

79. 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

80. US Food and Drug Administration. 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

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

82. 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

83. 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

84. 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

85. 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

86. 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

87. 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

88. 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

89. 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

90. 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.

91. 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

92. 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

93. 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

94. National Institute for Health and Care Excellence. Anaphylaxis: assessment and referral after emergency treatment. Aug 2020 [internet publication].Full text