Национальные клинические рекомендации по диагностике и лечению мастоцитоза

Введение. Рекомендации посвящены современному состоянию проблемы диагностики и лечения мастоцитоза.

Цель — предоставить консолидированное мнение российских экспертов по лечению взрослых больных мастоцитозом.

Основные сведения. Разработаны рекомендации с  учетом зарубежных публикаций и  отечественного опыта, на  основе мировых клинических исследований по  лечению системного мастоцитоза, кожного мастоцитоза, тучноклеточного лейкоза и других форм мастоцитоза. Показано значение молекулярно-генетического исследования костного мозга и  периферической крови на  наличие перестроек гена KITD816V. Приведены схемы лечения с применением мидостаурина, иматиниба, кладрибина, гидроксикарбамида, интерферон альфа, трансплантации гемопоэтических стволовых клеток. Приведен прогноз при различных формах мастоцитоза.

1. Valent P., Akin C., Hartmann K., et al. Advances in the classifi cation and treatment of mastocytosis: Current status and outlook toward the future. Cancer Res. 2017; 77(6): 1261–1270. DOI: 10.1158/0008-5472.CAN-16-2234.

2. Cohen S.S., Skovbo S., Vestergaard H., et al. Epidemiology of systemic mastocytosis in Denmark. Br J Haematol. 2014; 166(4): 521–528. DOI: 10.1111/bjh.12916.

3. Valent P., Akin C., Sperr W.R., et al. Diagnosis and treatment of systemic mastocytosis: State of the art. Br J Haematol. 2003. 122(5): 695–717. DOI: 10.1046/j.1365-2141.2003.04575.x.

4. Briley L.D., Phillips C.M. Cutaneous mastocytosis: A review focusing on the pediatric population. Clin Pediatr. 2008; 47(8): 757–761. DOI: 10.1177/0009922808318344.

5. Лебедева Т.Ю., Федерякина О.Б., Дубенский В.В., Катунина О.Р. Мастоцитоз у детей. Тверской медицинский журнал. 2014; 1: 48–61.

6. Lange M., Niedoszytko M., Renke J., Gleń J., Nedoszytko B. Clinical aspects of paediatric mastocytosis: A review of 101 cases. J Eur Acad Dermatol Venereol. 2013; 27(1): 97–102. DOI: 10.1111/j.1468-3083.2011.04365.x.

7. Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin North Am. 2014; 34(2): 283–295. DOI: 10.1016/j.iac.2014.01.003.

8. Koga H., Kokubo T., Akaishi M., Iida K., Korematsu S. Neonatal onset diffuse cutaneous mastocytosis: A case report and review of the literature. Pediatr Dermatol. 2011; 28(5): 542–546. DOI: 10.1111/j.1525-1470.2010.01212.x.

9. Horny H.P., Metcalfe D.D., Akin C., et al. Mastocytosis. In: Swerdlow S.H., et al. (eds). WHO Classifi cation of tumors of hematopoietic and lymphoid tissues. Lyon, France: International Agency for Research and Cancer (IARC); 2017: 62–69.

10. Sperr W.R., Escribano L., Jordan J.-H., et al. Morphologic properties of neoplastic mast cells: Delineation of stages of maturation and implication for cytological grading of mastocytosis. Leuk Res. 2001; 25(7): 529–536. DOI: 10.1016/s0145-2126(01)00041-8.

11. Valent P., Sillaber C., Bettelheim P. The growth and differentiation of mast cells. Prog Growth Factor Res. 1991; 3(1): 27–41. DOI: 10.1016/0955- 2235(91)90011-r.

12. Sperr W.R., Jordan J.H., Fiegl M., et al. Serum tryptase levels in patients with mastocytosis: Correlation with mast cell burden and implication for defining the category of disease. Int Arch Allerg Immunol. 2002; 128(2): 136–141. DOI: 10.1159/000059404.

13. Miettinen M., Lasota J. KIT (CD117): A review on expression in normal and neoplastic tissues, and mutations and their clinicopathologic correlation. Appl Immunohistohem Mol Morphol. 2005; 13(3): 205–220. DOI: 10.1097/01.pai.0000173054.83414.22.

14. Kristensen T., Vestergaard H., Møller M.B. Improved detection of the KIT D816V mutation in patients with systemic mastocytosis using a quantitative and highly sensitive real-time qPCR assay. J Mol Diagn. 2011; 13(2): 180–188. DOI: 10.1016/j.jmoldx.2010.10.004.

15. Cardet J.C., Akin C., Lee M.J. Mastocytosis: Update on pharmacotherapy and future directions. Expert Opin Pharmacother. 2013; 14(15): 2033–2045. DOI: 10.1517/14656566.2013.824424.

16. Metcalfe D.D. Mast cells and mastocytosis. Blood. 2008; 112(4): 946–956. DOI: 10.1182/blood-2007-11-078097.

17. Sotlar K., Escribano L., Landt O., et al. One-step detection of c-kit point mutations using peptide nucleic acid-mediated polymerase chain reaction clamping and hybridization probes. Am J Pathol. 2003; 162(3): 737–746. DOI: 10.1016/S0002-9440(10)63870-9.

18. Jawhar M., Schwaab J., Schnittger S., et al. Additional mutations in SRSF2, ASXL1 and/or RUNX1 identify a high-risk group of patients with KIT D816V(+) advanced systemic mastocytosis. Leukemia. 2016; 30(1): 136–143. DOI: 10.1038/leu.2015.284.

19. Puri N., Roche P.A. Mast cells possess distinct secretory granule subsets whose exocytosis is regulated by different SNARE isoforms. Proc Natl Acad Sci USA. 2008; 105(7): 2580–2585. DOI: 10.1073/pnas.0707854105.

20. Castells M., Metcalfe D.D., Escribano L. Diagnosis and treatment of cutaneous mastocytosis in children: Practical recommendations. Am J Clin Dermatol. 2011; 12(4): 259–270. DOI: 10.2165/11588890-000000000-00000.

21. Marrouche N., Grattan C. TMEP or not TMEP: That is the question. J Am Acad Dermatol. 2014; 70(3): 581–582. DOI: 10.1016/j.jaad.2013.09.006.

22. Vannorsdall E.J., Collins J.A., Chen Q.C., et al. Symptomatic response to imatinib mesylate in cutaneous mastocytosis associated with chronic myelomonocytic leukemia. Curr Oncol. 2013; 20(4): e349–e353. DOI: 10.3747/co.20.1301.

23. Berezowska S., Flaig M.J., Ruë ff F., et al. Adult-onset mastocytosis in the skin is highly suggestive of systemic mastocytosis. Mod Pathol. 2014; 27(1): 19–29. DOI: 10.1038/modpathol.2013.117.

24. Захарцева Л.М., Шатрова К.М., Крячок И.А. и др. Системный мастоцитоз с преимущественным поражением костной и лимфатической системы (обзор литературы и случай из практики). Онкология. 2015; 17(4): 236–242.

25. Alvarez-Twose I., Morgado J.M., Sá nchez-Muñ oz L., et al. Current state of biology and diagnosis of clonal mast cell diseases in adults. Int J Lab Hematol. 2012; 34(5): 445–460. DOI: 10.1111/j.1751-553X.2012.01427.x.

26. Синельникова Н.А., Калинина Н.М., Савенкова Н.Д. Хроническая крапивница в детском возрасте. Формы хронической крапивницы у детей. Дифференциальная диагностика (часть II). Медицинская иммунология. 2013; 15(4): 313–324.

27. Спирина В.Г., Мащенко Ю.В. Особенности течения пигментной крапивницы у детей. Бюллетень медицинских интернет-конференций . 2014; 4(4): 276.

28. Yankova R., Abadjieva T., Belovezhdov V. Cutaneous mastocytosis with persistent blistering: Successful treatment with methylprednisolone and 3-year follow-up management. Dermatol Ther. 2015; 5(2): 145–150. DOI: 10.1007/s13555-015-0073-6.

29. Pardanani A. Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratifi cation, and management. Am J Hematol. 2016; 91(11): 1146–1159. DOI: 10.1002/ajh.24553.

30. Bonadonna P., Pagani M., Aberer W., et al. Drug hypersensitivity in clonal mast cell disorders: ENDA/EAACI position paper. Allergy. 2015; 70(7): 755– 763. DOI: 10.1111/all.12617.

31. Меликян А.Л., Суборцева И.Н., Горячева С.Р., и др. Мастоцитоз (обзор литературы и описание клинических случаев). Терапевтический архив. 2014; 86(12): 127–134. DOI: 10.17116/terarkh20148612127-134.

32. Лебедева Т.Ю., Федерякина О.Б., Дубенский В.В. и др. Клинический случай мастоцитоза у новорожденного ребенка. Верхневолжский медицинский журнал. 2012; 10(4): 15–16.

33. Arock M., Akin C., Hermine O., Valent P. Current treatment options in patients with mastocytosis: Status in 2015 and future perspectives. Eur J Haematol. 2015; 94(6): 474–490. DOI: 10.1111/ejh.12544.

34. Lim K.H., Tefferi A., Lasho T.L., et al. Systemic mastocytosis in 342 consecutive adults: Survival studies and prognostic factors. Blood. 2009; 113(23): 5727– 5736. DOI: 10.1182/blood-2009-02-205237.

35. Pardanani A. How I treat patients with indolent and smoldering mastocytosis (rare conditions but difficult to manage). Blood. 2013; 121(16): 3085–3094. DOI: 10.1182/blood-2013-01-453183.

36. Matito A., Alvarez-Twose I., Morgado J.M., et al. Anaphylaxis as a clinical manifestation of clonal mast cell disorders. Curr Allergy Asthma Rep. 2014; 14(8): 450. DOI: 10.1007/s11882-014-0450-8.

37. van der Veer E., van der Goot W., de Monchy J.G., et al. High prevalence of fractures and osteoporosis in patients with indolent systemic mastocytosis. Allergy. 2012; 67(3): 431–438. DOI: 10.1111/j.1398-9995.2011.02780.x.

38. Hermine O., Lortholary O., Leventhal P.S., et al. Case-control cohort study of patients’ perceptions of disability in mastocytosis. PLoS One. 2008. 3(5): e2266. DOI: 10.1371/journal.pone.0002266.

39. Gülen T., Hägglund H., Dahlén B., Nilsson G. High prevalence of anaphylaxis in patients with systemic mastocytosis — a single-centre experience. Clin Exp Allergy. 2014; 44(1): 121–129. DOI: 10.1111/cea.12225.

40. Koenig M., More J., Reynaud J., Varvat C., Cathébras P. An unusual cause of spontaneous bleeding in the intensive care unit — mastocytosis: A case report. Cases J. 2008; 1(1): 100. DOI: 10.1186/1757-1626-1-100.

41. Carvalhosa A.B., Aouba A., Damaj G., et al. A French national survey on clotting disorders in mastocytosis. Medicine. 2015; 94(40): e1414. DOI: 10.1097/MD.0000000000001414.

42. Weiler C.R., Akin C. Accurate Diagnosis and Prognosis in Systemic Mastocytosis: The Role of Mutational Analysis. J Allergy Clin Immunol Pract. 2020; 8(9): 3128–3129. DOI: 10.1016/j.jaip.2020.06.030.

43. Pardanani A., Baek J.Y., Li C.Y., at al. Systemic mast cell disease without associated hematologic disorder: A combined retrospective and prospective study. Mayo Clin Proc. 2002; 77(11): 1169–1175. DOI: 10.4065/77.11.1169.

44. Kovalszki A., Weller P.F. Eosinophilia in mast cell disease. Immunol Allergy Clin North Am. 2014; 34(2): 357–364. DOI: 10.1016/j.iac.2014.01.013.

45. Travis W.D., Li C.Y., Bergstralh E.J., Yam L.T., Swee R.G. Systemic mast cell disease. Analysis of 58 cases and literature review. Medicine. 1988; 67(6): 345–368.

46. Pardanani A., Brockman S.R., Paternoster S.F., et al. FIP1L1-PDGFRA fusion: Prevalence and clinicopathologic correlates in 89 consecutive patients with moderate to severe eosinophilia. Blood. 2004; 104(10): 3038–3045. DOI: 10.1182/blood-2004-03-0787.

47. Pardanani A., Ketterling R.P., Brockman S.R., et al. CHIC2 deletion, a surrogate for FIP1L1-PDGFRA fusion, occurs in systemic mastocytosis associated with eosinophilia and predicts response to imatinib mesylate therapy. Blood. 2003; 102(9): 3093–3096. DOI: 10.1182/blood-2003-05-1627.

48. Абдулкадыров К.М., Шуваев В.А., Мартынкевич И.С. Миелопролиферативные новообразования. Санкт-Петербург: Литтерра; 2016: 298 с.

49. Horny H.P., Sotlar K., Sperr W.R., Valent P. Systemic mastocytosis with associated clonal haematological non-mast cell lineage diseases: A histopathological challenge. J Clin Pathol. 2004; 57(6): 604–608. DOI: 10.1136/jcp.2003.014860.

50. Horny H.P., Valent P. Histopathological and immunohistochemical aspects of mastocytosis. Int Arch Allergy Immunol. 2002; 127(2): 115–117. DOI: 10.1159/000048180.

51. Akin C. Molecular diagnosis of mast cell disorders: A paper from the 2005 William Beaumont Hospital symposium on molecular pathology. J Mol Diagn. 2006; 8(4): 412–419. DOI: 10.2353/jmoldx.2006.060022.

52. Jawhar M., Schwaab J., Hausmann D., et al. Splenomegaly, elevated alkaline phosphatase and mutations in the SRSF2/ASXL1/RUNX1 gene panel are strong adverse prognostic markers in patients with systemic mastocytosis. Leukemia. 2016; 30(12): 2342–2350. DOI: 10.1038/leu.2016.190.

53. Sperr W.R., Kundi M., Alvarez-Twose I., et al. International prognostic scoring system for mastocytosis (IPSM): A retrospective cohort study. Lancet Haematol. 2019; 6(12): e638–e649. DOI: 10.1016/S2352-3026(19)30166-8.

54. Pardanani A., Shah S., Mannelli F., et al. Mayo alliance prognostic system for mastocytosis: Clinical and hybrid clinical-molecular models. Blood Adv. 2018; 2(21): 2964–2972. DOI: 10.1182/bloodadvances.2018026245.

55. Jawhar M., Schwaab J., Álvarez-Twose I., et al. Mutation-adjusted risk score for advanced systemic mastocytosis. J Clin Oncol. 2019; 37(31): 2846–2856. DOI: 10.1200/JCO.19.00640.

56. Siebenhaar F., von Tschirnhaus E., Hartmann K., et al. Development and validation of the mastocytosis quality of life questionnaire: MC-QoL. Allergy. 2016; 71(6): 869–877. DOI: 10.1111/all.12842.

57. 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; 137(1): 35–45. DOI: 10.1016/j.jaci.2015.08.034.

58. Mashiah J., Harel A., Bodemer C., et al. Topical pimecrolimus for paediatric cutaneous mastocytosis. Clin Exp Dermatol. 2018; 43(5): 559–565. DOI: 10.1111/ced.13391.

59. Brazzelli V., Grassi S., Merante S., et al. Narrow-band UVB phototherapy and psoralen-ultraviolet A photochemotherapy in the treatment of cutaneous mastocytosis: A study in 20 patients. Photodermatol Photoimmunol Photomed. 2016; 32(5–6): 238–246. DOI: 10.1111/phpp.12248.

60. Sotiriou E., Apalla Z., Ioannides D. Telangiectasia macularis eruptive perstans successfully treated with PUVA therapy. Photodermatol Photoimmunol Photomed. 2010; 26(1): 46–47. DOI: 10.1111/j.1600-0781.2009.00480.x.

61. Pyatilova P., Teplyuk N., Olisova O., et al. Effi cacy of PUVA in Russian patients with mastocytosis: A case series and review of literature. Int J Dermatol. 2018; 57(9): e59–e64. DOI: 10.1111/ijd.14103.

62. Dewachter P., Castells M.C., Hepner D.L., Mouton-Faivre C. Perioperative management of patients with mastocytosis. Anesthesiology. 2014; 120(3): 753– 759. DOI: 10.1097/ALN.0000000000000031.

63. Brazzelli V., Grasso V., Manna G., et al. Indolent systemic mastocytosis treated with narrow-band UVB phototherapy: Study of five cases. J Eur Acad Dermatol Venereol. 2012; 26(4): 465–469. DOI: 10.1111/j.1468-3083.2011.04098.x.

64. van Anrooij B., Elberink J.N.G.O., Span L.F.R., et al. Midostaurin in patients with indolent systemic mastocytosis: An open-label phase 2 trial. J Allergy Clin Immunol. 2018; 142(3): 1006–1008e7. DOI: 10.1016/j.jaci.2018.06.003.

65. Gleixner K.V., Mayerhofer M., Aichberger K.J., et al. PKC412 inhibits in vitro growth of neoplastic human mast cells expressing the D816V-mutated variant of KIT: Comparison with AMN107, imatinib, and cladribine (2CdA) and evaluation of cooperative drug effects. Blood. 2006; 107(2): 752–759. DOI: 10.1182/blood-2005-07-3022.

66. Gotlib J., Kluin-Nelemans H.C., George T.I., et al. Efficacy and safety of midostaurin in advanced systemic mastocytosis. N Engl J Med. 2016; 374(26): 2530–2541. DOI: 10.1056/NEJMoa1513098.

67. Pardanani A., Hoffbrand A.V., Butterfi eld J.H., Tefferi A. Treatment of systemic mast cell disease with 2-chlorodeoxyadenosine. Leuk Res. 2004; 28(2): 127–131. DOI: 10.1016/s0145-2126(03)00185-1.

68. Lim K.H., Pardanani A., Butterfi eld J.H., Li C.-Y., Tefferi A. Cytoreductive therapy in 108 adults with systemic mastocytosis: Outcome analysis and response prediction during treatment with interferon-alpha, hydroxyurea, imatinib mesylate or 2-chlorodeoxyadenosine. Am J Hematol. 2009; 84(12): 790–794. DOI: 10.1002/ajh.21561.

69. Kluin-Nelemans H.C., Jansen J.H., Breukelman H., et al. Response to interferon alfa-2b in a patient with systemic mastocytosis. N Engl J Med. 1992; 326(9): 619–623. DOI: 10.1056/NEJM199202273260907.

70. Hauswirth A.W., Simonitsch-Klupp I., Uffmann M., et al. Response to therapy with interferon alpha-2b and prednisolone in aggressive systemic mastocytosis: Report of five cases and review of the literature. Leuk Res. 2004; 28(3): 249–257. DOI: 10.1016/s0145-2126(03)00259-5.

71. Lippert U., Henz B.M. Long-term effect of interferon alpha treatment in mastocytosis. Br J Dermatol. 1996; 134(6): 1164–1165.

72. Simon J., Lortholary O., Caillat-Vigneron N., et al. Interest of interferon alpha in systemic mastocytosis. The French experience and review of the literature. Pathol Biol. 2004; 52(5): 294–299. DOI: 10.1016/j.patbio.2004.04.012.

73. Akin C., Brockow K., D’Ambrosio C., et al. Effects of tyrosine kinase inhibitor STI571 on human mast cells bearing wild-type or mutated c-kit. Exp Hematol. 2003; 31(8): 686–692. DOI: 10.1016/s0301-472x(03)00112-7.

74. Droogendijk H.J., Kluin-Nelemans H.J., van Doormaal J.J., et al. Imatinib mesylate in the treatment of systemic mastocytosis: A phase II trial. Cancer. 2006; 107(2): 345–351. DOI: 10.1002/cncr.21996.

75. Verstovsek S., Tefferi A., Cortes J., et al. Phase II study of dasatinib in philadelphia chromosome-negative acute and chronic myeloid diseases, including systemic mastocytosis. Clin Cancer Res. 2008; 14(12): 3906–3915. DOI: 10.1158/1078-0432.CCR-08-0366.

76. Gotlib J., Baird J.H., George T.I., et al. A phase 2 study of brentuximab vedotin in patients with CD30-positive advanced systemic mastocytosis. Blood Adv. 2019; 3(15): 2264–2271. DOI: 10.1182/bloodadvances.2019000152.

77. Ustun C., Gotlib J., Popat U., et al. Consensus opinion on allogeneic hematopoietic cell transplantation in advanced systemic mastocytosis. Biol Blood Marrow Transplant. 2016; 22(8): 1348–1356. DOI: 10.1016/j.bbmt.2016.04.018.

78. Ustun C., Reiter A., Scott B.L., et al. Hematopoietic stem-cell transplantation for advanced systemic mastocytosis. J Clinical Oncol. 2014; 32(29): 3264–3274. DOI: 10.1200/JCO.2014.55.2018.

79. Pardanani A. Systemic mastocytosis in adults: 2019 update on diagnosis, risk stratification and management. Am J Hematol. 2019; 94(3): 363–377. DOI: 10.1002/ajh.25371.

80. Gotlib J., Gerds A.T., Bose P., et al. Systemic mastocytosis, version 2.2019, NCCN clinical practice guidelines in oncology. J Natl Compr Canc Netw. 2018; 16(12): 1500–1537. DOI: 10.6004/jnccn.2018.0088.

81. Martynova A., Nael A., O`Neill C., et al. Aggressive systemic mastocytosis: midostaurin is safe, feasible and associated with durable response post-haploidentical allogeneic stem cell transplant. Brit J Hematol. 2019; 186(5): 139–141. DOI: 10.1111/bjh.15980.

82. DeAngelo D.J., George T.I., Linder A., et al. Efficacy and safety of midostaurin in patients with advanced systemic mastocytosis: 10-year median follow-up of a phase II trial. Leukemia. 2018; 32(2): 470–478. DOI: 10.1038/leu.2017.234.

83. Wimazal F., Schwarzmeier J., Sotlar K., et al. Splenic mastocytosis: Report of two cases and detection of the transforming somatic C-KIT mutation D816V. Leuk Lymphoma. 2004; 45(4): 723–729. DOI: 10.1080/1042819032000140979.

84. Friedman B., Darling G., Norton J., Hamby L., Metcalfe D. Splenectomy in the management of systemic mast cell disease. Surgery. 1990; 107(1): 94–100.

85. Gotlib J., Pardanani A., Akin C., et al. International working group – Myeloproliferative neoplasms research and treatment (IWG-MRT) & European competence network on Mastocytosis (ECNM) consensus response criteria in advanced systemic mastocytosis. Blood. 2013; 121(13): 2393–2401. DOI: 10.1182/blood-2012-09-458521.

86. The Common Terminology Criteria for Adverse Events (CTCAE). URL: https://ctep.cancer.gov/protocoldevelopment/electronic_applications/ctc.htm

87. Matito A., Morgado J.M., Sánchez-López P., et al. Management of anesthesia in adult and pediatric mastocytosis: A study of the Spanish Network on Mastocytosis (REMA) based on 726 anesthetic procedures. Int Arch Allergy Immunol. 2015; 167(1): 47–56. DOI: 10.1159/000436969.

88. Mertes P.M., Volcheck G.W., Garvey L.H., et al. Epidemiology of perioperative anaphylaxis. Presse Med. 2016; 45(9): 758–767. DOI: 10.1016/j.lpm.2016.02.024.

89. Palmiere C., Bonetti L.R. Risk factors in fatal cases of anaphylaxis due to contrast media: A forensic evaluation. Int Arch Allergy Immunol. 2014; 164(4): 280– 288. DOI: 10.1159/000366204.

90. Thong B., Tan T. Epidemiology and risk factors for drug allergy. Br J Clin Pharmacol. 2011; 71(5): 684–700. DOI: 10.1111/j.1365-2125.2010.03774.x.

91. Andreucci M., Solomon R., Tasanarong A. Side effects of radiographic contrast media: Pathogenesis, risk factors, and prevention. Biomed Res Int. 2014; 2014: 741018. DOI: 10.1155/2014/741018.

92. 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(2): 226–232. DOI: 10.1111/j.1398-9995.2007.01569.x.

93. González de Olano D., de la Hoz Caballer B., Núñez López R., et al. Prevalence of allergy and anaphylactic symptoms in 210 adult and pediatric patients with mastocytosis in Spain: A study of the Spanish network on mastocytosis (REMA). Clin Exp Allergy. 2007; 37(10): 1547–1555. DOI: 10.1111/j.1365-2222.2007.02804.x.

94. Gülen T., Hägglund H., Dahlén B., Nilsson G. High prevalence of anaphylaxis in patients with systemic mastocytosis — a single-centre experience. Clin Exp Allergy. 2014; 44: 121–129. DOI: 10.1111/cea.12225.

95. Hermans M., Rietveld M., van Laar J., et al. Systemic mastocytosis: A cohort study on clinical characteristics of 136 patients in a large tertiary centre. Eur J Intern Med. 2016; 30: 25–30. DOI: 10.1016/j.ejim.2016.01.005.

96. Matito A., Álvarez-Twose I., Morgado J.M., et al. Clinical impact of pregnancy in mastocytosis: A study of the Spanish network on mastocytosis (REMA) in 45 cases. Int Arch Allergy Immunol. 2011; 156(1): 104–111. DOI: 10.1159/000321954.

97. Simons F., Schatz M. Anaphylaxis during pregnancy. J Allergy Clin Immunol. 2012; 130(3): 597–606. DOI: 10.1016/j.jaci.2012.06.035.

98. Ulbrich F., Engelstädter H., Wittau N., Steinmann D. Anaesthetic management of emergency caesarean section in a parturient with systemic mastocytosis. Int J Obstet Anesth. 2013; 22(3): 243–246. DOI: 10.1016/j.ijoa.2013.03.011.

99. Konrad F.M., Schroeder T.H. Anaesthesia in patients with mastocytosis. Acta Anaesth Scand. 2009; 53(2): 270–271. DOI: 10.1111/j.1399-6576.2008.01780.x.

100. Hermans M.A.W., Arends N.J.T., van Wijk R.G., et al. Management around invasive procedures in mastocytosis: An update. Ann Allergy Asthma Immunol. 2017; 119(4): 304–309. DOI: 10.1016/j.anai.2017.07.022.

101. Unterbuchner C., Hierl M., Seyfried T., Metterlein T. Anaesthesia and orphan disease: Rapid sequence induction in systemic mastocytosis. Eur J Anaesthesiol. 2017; 34(3): 176–178. DOI: 10.1097/EJA.0000000000000568.