Mastocitose sistêmica
visão do dermatologista e relato de um caso associado a líquen plano
Palavras-chave:
Mastocitose, mastocitose sistêmica, líquen plano, mastócitos, mutação com ganho de funçãoResumo
Contexto: A mastocitose ocorre devido a uma proliferação neoplásica e clonal de mastócitos que se acumulam em um ou mais sistemas de órgãos. A doença é heterogênea, com manifestações que vão desde lesões cutâneas que podem regredir espontaneamente até ne- oplasias altamente agressivas associadas à falência de múltiplos órgãos e baixa sobrevida. Não há relatos na literatura de sua associação com líquen plano. Descrição do caso: Relatamos o caso de uma paciente com diagnóstico de mastocitose sistêmica agressiva que apresentou durante o acompanhamento quadro compatível com líquen plano. Discussão: O diagnóstico da mastocitose sistêmica é baseado em critérios que foram refinados recentemente. O tratamento classicamente envolve bloqueio de mediadores de mastócitos e terapia citorredutora para variantes avançadas da doença. Novas drogas como a midostaurina e o avapritinibe são promissoras. Conclusões: Mesmo não fazendo parte da rotina do dermatologista, a mastocitose sistêmica deve ser uma doença lembrada pelo acometimento da pele e potencial gravidade do quadro.
Referências
Horny HP, Akin C, Arber DA, et al. Mastocytosis. In Swerdlow SH, Campo E, Harris NL, et al, editors. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues; Revised 4th ed. Lyon: IARC; 2017. p. 61–69.
Brockow K. Epidemiology, prognosis, and risk factors in mastocytosis. Immunol Allergy Clin North Am. 2014;34(2):283- 95. PMID: 24745674; https://doi.org/10.1016/j.iac.2014.01.003.
Fernandes IC, Teixeira Mdos A, Freitas I, et al. Adult mastocytosis: a review of the Santo António Hospital ‘s experience and anevaluation of World Health Organization criteria for the diagnosis of systemic disease. An Bras Dermatol. 2014;89(1):59-66. PMID:24626649; https://doi.org/10.1590/abd1806-4841.20141847.
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. PMID: 26476479; https://doi.org/10.1016/j.jaci.2015.08.034.
Pardanani A. Systemic mastocytosis in adults: 2021 Update on diagnosis, risk stratification and management. Am J Hematol. 2021;96(4):508-25. PMID: 33524167; https://doi.org/10.1002/ajh.26118.
Metcalfe DD. Mast cells and mastocytosis. Blood. 2008;112(4):946-56. PMID: 18684881; https://doi.org/10.1182/blood-2007-11-078097.
Carter MC, Metcalfe DD, Komarow HD. Mastocytosis. Immunol Allergy Clin North Am. 2014;34(1):181-96. PMID: 24262698; https://doi.org/10.1016/j.iac.2013.09.001.
Khoury JD, Solary E, Abla O, et al. The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms. Leukemia. 2022;36(7):1703-19. PMID: 35732831; https://doi.org/10.1038/s41375-022-01613-1.9.
Velloso EDRP, Padulla GA, de Cerqueira AMM, et al. Diagnosis and treatment of systemic mastocytosis in Brazil: Recommendations of a multidisciplinary expert panel. Hematol Transfus Cell Ther. 2022:S2531-1379(22)00080-3. PMID:35688791; https://doi.org/10.1016/j.htct.2022.04.006.
Tziotzios C, Lee JYW, Brier T, et al. Lichen planus and lichenoid dermatoses: Clinical overview and molecular basis. J Am Acad Dermatol. 2018;79(5):789-804. PMID: 30318136; https://doi.org/10.1016/j.jaad.2018.02.010.
Ferreira S, Fernandes I, Cabral R, et al. Manifestações Cutâneas nas Mastocitoses: Atualização [Cutaneous Manifestations in Mastocytosis: Update]. Acta Med Port. 2020;33(4):275-81. PMID: 32238242; https://doi.org/10.20344/amp.12189.
Reiter A, George TI, Gotlib J. New developments in diagnosis, prognostication, and treatment of advanced systemic mastocytosis. Blood. 2020;135(16):1365-76. PMID: 32106312; https://doi.org/10.1182/blood.2019000932.
Scherber RM, Borate U. How we diagnose and treat systemic mastocytosis in adults. Br J Haematol. 2018;180(1):11-23. PMID:29048112; https://doi.org/10.1111/bjh.14967.
Theoharides TC, Valent P, Akin C. Mast Cells, Mastocytosis, and Related Disorders. N Engl J Med. 2015;373(2):163-72. PMID: 26154789; https://doi.org/10.1056/NEJMra1409760.
Sciumè M, De Magistris C, Galli N, et al. Target Therapies for Systemic Mastocytosis: An Update. Pharmaceuticals (Basel). 2022;15(6):738. PMID: 35745657; https://doi.org/10.3390/ph15060738.
Giannetti MP. Treatment of systemic mastocytosis: Novel and emerging therapies. Ann Allergy Asthma Immunol. 2021;127(4):412-9. PMID: 34216794; https://doi.org/10.1016/j.anai.2021.06.021.
El Hussein S, Chifotides HT, Khoury JD, Verstovsek S, Thakral B. Systemic Mastocytosis and Other Entities Involving Mast Cells: A Practical Review and Update. Cancers (Basel). 2022;14(14):3474. PMID: 35884535; https://doi.org/10.3390/cancers14143474.
Arock M, Sotlar K, Akin C, et al. KIT mutation analysis in mast cell neoplasms: recommendations of the European Competence Network on Mastocytosis. Leukemia. 2015;29(6):1223-32. PMID: 25650093; https://doi.org/10.1038/leu.2015.24.
Gotlib J, Kluin-Nelemans HC, George TI, et al. Efficacy and Safety of Midostaurin in Advanced Systemic Mastocytosis. N Engl J Med. 2016;374(26):2530-41. PMID: 27355533; https://doi.org/10.1056/NEJMoa1513098.
Valent P, Akin C, Metcalfe DD. Mastocytosis: 2016 updated WHO classification and novel emerging treatment concepts. Blood. 2017;129(11):1420-7. PMID: 28031180; https://doi.org/10.1182/blood-2016-09-731893.
Le Cleach L, Chosidow O. Clinical practice. Lichen planus. N Engl J Med. 2012;366(8):723-32. PMID: 22356325; https://doi.org/10.1056/NEJMcp1103641.
Martínez de Lagrán Z, González-Hermosa MA, Blaya-Alvarez B, Díaz-Pérez JL. Un caso de liquen plano asociado a tratamiento con imatinib [A case of lichen planus associated to treatment with imatinib]. Med Clin (Barc). 2009;133(20):805-6. PMID: 19243795; https://doi.org/10.1016/j.medcli.2008.11.010.
Sudha R, Vetrichevvel TP, Krishnarathnam K, Anandan S. Imatinib induced lichen planus. Indian J Dermatol. 2011;56(3):351-2. PMID: 21772613; https://doi.org/10.4103/0019-5154.82502.
Roux C, Boisseau-Garsaud AM, Saint-Cyr I, et al. Toxidermie lichénoïde à l’imatinib (Glivec) [Lichenoid cutaneous reaction to imatinib]. Ann Dermatol Venereol. 2004;131(6-7 Pt 1):571-3. PMID: 15318140; https://doi.org/10.1016/s0151-9638(04)93669-1.
Protzer U, Ochsendorf FR, Leopolder-Ochsendorf A, Holtermüller KH. Exacerbation of lichen planus during interferon alfa-2a therapy for chronic active hepatitis C. Gastroenterology. 1993;104(3):903-5. PMID: 8440441; https://doi.org/10.1016/0016-5085(93)91029-h.
