Diagnosis and treatment of systemic mastocytosis in Brazil: Recommendations of a multidisciplinary expert panel

Elvira D Rodrigues Pereira Velloso; Geórgia A Padulla; Ana Maria Mósca de Cerqueira; Adriana Martins de Sousa; Alex Freire Sandes; Fabiola Traina; Fernanda Salles Seguro; Frederico Lisboa Nogueira; Grazielly de Fátima Pereira; José Laerte Boechat; Katia Borgia Barbosa Pagnano; Luan Lima Marchi; Luis Felipe Ensina; Mara Giavina-Bianchi; Marcelo Vivolo Aun; Rosana Câmara Agondi; Fabio Pires de Souza Santos; Pedro Giavina-Bianchi

doi:10.1016/j.htct.2022.04.006

Diagnosis and treatment of systemic mastocytosis in Brazil: Recommendations of a multidisciplinary expert panel

Hematol Transfus Cell Ther. 2022 Oct-Dec;44(4):582-594. doi: 10.1016/j.htct.2022.04.006. Epub 2022 May 26.

Authors

Elvira D Rodrigues Pereira Velloso¹, Geórgia A Padulla², Ana Maria Mósca de Cerqueira³, Adriana Martins de Sousa⁴, Alex Freire Sandes⁵, Fabiola Traina⁶, Fernanda Salles Seguro², Frederico Lisboa Nogueira⁷, Grazielly de Fátima Pereira⁸, José Laerte Boechat⁹, Katia Borgia Barbosa Pagnano¹⁰, Luan Lima Marchi², Luis Felipe Ensina¹¹, Mara Giavina-Bianchi², Marcelo Vivolo Aun¹², Rosana Câmara Agondi², Fabio Pires de Souza Santos¹³, Pedro Giavina-Bianchi²

Affiliations

¹ Clinical Immunology and Allergy Division. Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil; Hospital Israelita Albert Einstein, São Paulo, SP, Brazil. Electronic address: elvira.velloso@fm.usp.br.
² Clinical Immunology and Allergy Division. Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil.
³ Hospital Municipal Jesus, Rio de Janeiro, RJ, Brazil.
⁴ Instituto de Pediatria e Puericultura Martagão Gesteira da Universidade Federal do Rio de Janeiro (IPPMG UFRJ), Rio de Janeiro, RJ, Brazil.
⁵ Grupo Fleury, São Paulo, SP, Brazil; Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
⁶ Faculdade de Medicina de Ribeirão Preto, Universidade de São Paulo (FMRP USP), São Paulo, SP, Brazil.
⁷ Hospital Luxemburgo, Instituto Mário Penna, Belo Horizonte, MG, Brazil; Grupo Oncoclínicas, Belo Horizonte, MG, Brazil.
⁸ Hospital das Clínicas da Universidade Federal do Paraná (HC UFPR), Curitiba, PR, Brazil.
⁹ Faculdade de Medicina da Universidade Federal Fluminense (FM UFF), Niteroi, RJ, Brazil.
¹⁰ Hemocentro da Universidade Estadual de Campinas (Unicamp), Campinas, SP, Brazil.
¹¹ Universidade Federal de São Paulo (Unifesp), São Paulo, SP, Brazil.
¹² Clinical Immunology and Allergy Division. Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP, Brazil; Faculdade Israelita de Ciências da Saúde Albert Einstein, São Paulo, SP, Brazil.
¹³ Hospital Israelita Albert Einstein, São Paulo, SP, Brazil; A Beneficência Portuguesa de São Paulo, BP, São Paulo, SP, Brazil.

Abstract

Introduction: Systemic Mastocytosis comprises a group of neoplastic diseases characterized by clonal expansion and infiltration of mast cells into several organs. The diagnosis and treatment of this disease may be challenging for non-specialists.

Objective: Make suggestions or recommendations in Systemic Mastocytosis based in a panel of Brazilian specialists.

Method and results: An online expert panel with 18 multidisciplinary specialists was convened to propose recommendations on the diagnosis and treatment of Systemic Mastocytosis in Brazil. Recommendations were based on discussions of topics and multiple-choice questions and were graded using the Oxford Centre for Evidence-Based Medicine 2011 Levels of Evidence Chart.

Conclusion: Twenty-two recommendations or suggestions were proposed based on a literature review and graded according to the findings.

Keywords: Adults; Children; Diagnosis; Expert panel; Mastocytosis; Treatment.