Assessment of skin manifestations under mastocytosis in adults and their associations with symptoms, level of tryptase and subtype of the disease
Abstract
Clinical features of 30 adult patients suffering from mastocytosis with skin lesion were compared with the duration of the disease, severity of skin and systemic symptoms, involvement of the internals in the pathologic process, level of tryptase in the blood serum and presence of marrow lesion. Presence of skin and systemic symptoms, as well as the level of tryptase, can be useful for assessment of internal lesion in mastocytosis in adults.
About the Authors
N. P. Teplyuk
ФГАОУ ВО Первый МГМУ им. И. М. Сеченова МЗ РФ
Россия
Россия
P. M. Pyatilova
ФГАОУ ВО Первый МГМУ им. И. М. Сеченова МЗ РФ
Россия
Россия
References
1. Valent P. et al. Diagnostic criteria and classification of mastocytosis: a consensus proposal // Leuk Res. 2001. 25 (7): p. 603–625.
2. Valent, P. et al. Advances in the Classification and Treatment of Mastocytosis: Current Status and Outlook toward the Future // Cancer Res. 2017. 77 (6): p. 1261–1270.
3. Horny H. P., Parwaresch M. R., Lennert K. Bone marrow findings in systemic mastocytosis // Hum Pathol. 1985. 16 (8): p. 808–14.
4. Valent P. et al. Diagnosis and treatment of systemic mastocytosis: state of the art // Br J Haematol. 2003. 122 (5): p. 695–717.
5. Metcalfe D. D. Mast cells and mastocytosis // Blood. 2008. 112 (4): p. 946–956.
6. Parwaresch M. R., Horny H. P., Lennert K. Tissue mast cells in health and disease // Pathol Res Pract. 1985. 179 (4–5): p. 439–461.
7. Bodemer C. et al. Pediatric mastocytosis is a clonal disease associated with D816 V and other activating c-KIT mutations // J Invest Dermatol. 2010. 130 (3): p. 804–815.
8. Garcia-Montero A. C. et al. KIT mutation in mast cells and other bone marrow hematopoietic cell lineages in systemic mast cell disorders: a prospective study of the Spanish Network on Mastocytosis (REMA) in a series of 113 patients // Blood. 2006. 108 (7): p. 2366–2372.
9. Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis // Immunol Allergy Clin North Am. 2014. 34 (2): p. 283–295.
10. Hartmann K. 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): p. 35–45.
11. De la Hoz B. et al. Guidelines for the diagnosis, treatment and management of mastocytosis // An Sist Sanit Navar. 2008. 31 (1): p. 11–32.
12. Matito A. 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): p. 104–111.
13. Meni C. et al. Paediatric mastocytosis: a systematic review of 1747 cases // Br J Dermatol. 2015. 172 (3): p. 642–651.
14. Fett N. M., Teng J., Longley B. J. Familial urticaria pigmentosa: report of a family and review of the role of KIT mutations // Am J Dermatopathol. 2013. 35 (1): p. 113–116.
15. Hartmann K., Metcalfe D. D. Pediatric mastocytosis // Hematol Oncol Clin North Am. 2000. 14 (3): p. 625–640.
16. Wiechers T. et al. Large maculopapular cutaneous lesions are associated with favorable outcome in childhood-onset mastocytosis // J Allergy Clin Immunol. 2015. 136 (6): p. 1581–1590 e3.
17. Akin C., Valent P. Diagnostic criteria and classification of mastocytosis in 2014 // Immunol Allergy Clin North Am. 2014. 34 (2): p. 207–218.
18. Valent P. et al. European Competence Network on Mastocytosis (ECNM): 10-year jubilee, update, and future perspectives // Wien Klin Wochenschr. 2012. 124 (23–24): p. 807–814.
19. Valent P. et al. Standards and standardization in mastocytosis: consensus statements on diagnostics, treatment recommendations and response criteria // Eur J Clin Invest. 2007. 37 (6): p. 435–53.
20. Arber D. A. et al. The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia // Blood. 2016. 127 (20): p. 2391–2405.
21. Williams K. W. et al. Telangiectasia macularis eruptiva perstans or highly vascularized urticaria pigmentosa? // J Allergy Clin Immunol Pract. 2014. 2 (6): p. 813–815.
22. Brockow K. et al. Assessment of the extent of cutaneous involvement in children and adults with mastocytosis: relationship to symptomatology, tryptase levels, and bone marrow pathology // J Am Acad Dermatol. 2003. 48 (4): p. 508–516.
23. Galen B. T., Rose M. G. Darier’s sign in mastocytosis // Blood. 2014. 123 (8): p. 1127.
24. Skrabs C. C. Darier sign: a historical note // Arch Dermatol. 2002. 138 (9): p. 1253–1254.
25. Lange M. et al. Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature // J Eur Acad Dermatol Venereol. 2012. 26 (12): p. 1565–1571.
26. Neri I. et al. Diffuse cutaneous mastocytosis: a heterogeneous disease // Arch Dis Child, 2013. 98 (8): p. 607.
27. Wawrzycki B. et al. Diffuse cutaneous bullous mastocytosis in a newborn // Dermatol Ther. 2013. 26 (2): p. 176–179.
28. Jogie-Brahim S. et al. Expression of alpha-tryptase and beta-tryptase by human basophils // J Allergy Clin Immunol. 2004. 113 (6): p. 1086–1092.
29. Schwartz L. B. et al. Development of a new, more sensitive immunoassay for human tryptase: use in systemic anaphylaxis // J Clin Immunol. 1994. 14 (3): p. 190–204.
30. Escribano L. et al. Prognosis in adult indolent systemic mastocytosis: a long-term study of the Spanish Network on Mastocytosis in a series of 145 patients // J Allergy Clin Immunol. 2009. 124 (3): p. 514–521.
31. Chan I. J., Tharp M. D. Comparison of lesional skin c-KIT mutations with clinical phenotype in patients with mastocytosis // Clin Exp Dermatol. 2018.
32. Valent P. et al. Aggressive systemic mastocytosis and related mast cell disorders: current treatment options and proposed response criteria // Leuk Res. 2003. 27 (7): p. 635–641.
33. Sousa-Pinto B., Fonseca J. A., Gomes E. R. Frequency of self-reported drug allergy: A systematic review and meta-analysis with meta-regression // Ann Allergy Asthma Immunol. 2017. 119 (4): p. 362–373 e2.
34. Gomes E. R., Demoly P. Epidemiology of hypersensitivity drug reactions // Curr Opin Allergy Clin Immunol. 2005. 5 (4): p. 309–316.
35. Passali D. et al. The International Study of the Allergic Rhinitis Survey: outcomes from 4 geographical regions // Asia Pac Allergy. 2018. 8 (1): p. e7.
36. Tejedor Alonso M. A., Moro Moro M., Mugica Garcia M. V. Epidemiology of anaphylaxis // Clin Exp Allergy. 2015. 45 (6): p. 1027–1039.
37. Gorska A. et al. Risk factors for anaphylaxis in patients with mastocytosis // Pol Arch Med Wewn. 2015. 125 (1–2): p. 46–53.
Review
For citations:
Teplyuk N.P., Pyatilova P.M. Assessment of skin manifestations under mastocytosis in adults and their associations with symptoms, level of tryptase and subtype of the disease. Lechaschi Vrach. 2018;(10):91. (In Russ.)
Views:
155
JATS XML
Email this article 
