How scientific evidence can contribute to improve the diagnosis and comprehensive care of patients with TEMPI syndrome: A scoping review protocol

Authors

DOI:

https://doi.org/10.33448/rsd-v14i3.48388

Keywords:

Plasma cell dyscrasia, Erythrocytosis, Monoclonal gammopathies, Research protocol, TEMPI Syndrome.

Abstract

Objective: To document the planning and methodological processes of a scoping review protocol aimed at mapping the current scientific knowledge on TEMPI Syndrome. Introduction: TEMPI Syndrome is a rare, acquired, and multisystemic disease characterized by telangiectasias, elevated erythropoietin levels and erythrocytosis, monoclonal gammopathy, perinephric fluid collections, and intrapulmonary shunting. Justification: Due to its rarity, we hypothesize that the lack of awareness regarding this syndrome may lead to misdiagnosis or delayed diagnosis. A systematic literature review can contribute to increasing knowledge and dissemination of information on the topic. Methodology: The protocol was registered in the Open Science Framework and structured following the five-stage methodological framework by Arksey & O’Malley (2005), in accordance with the Joanna Briggs Institute guidelines. The review is guided by the question: "How can the available scientific evidence contribute to improving the diagnosis and comprehensive care of patients with TEMPI Syndrome?" A systematic search will be conducted in the Medical Literature Analysis and Retrieval System (MEDLINE) and in the Biblioteca Virtual em Saúde (BVS), without time restrictions. Gray literature will be retrieved using Google Scholar. Two independent reviewers will screen studies for eligibility. Results: Findings will be reported in accordance with the PRISMA-ScR (Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Reviews) and presented in tabular and narrative formats aligned with the study objectives and research question.

Downloads

Download data is not yet available.

References

Arksey, H., & O'Malley, L. (2005). Scoping studies: towards a methodological framework. International Journal of Social Research Methodology, 8, 19-32. 10.1080/1364557032000119616

Faria, Rosa Malena D., and Roberta O. Paula e Silva. 2007. “Gamopatias monoclonais: critérios diagnósticos e diagnósticos diferenciais.” Revista Brasileira de Hematologia e Hemoterapia 29:17–22. doi: https://doi.org/10.1590/S1516-84842007000100005.

Harari, Sergio. 2016. “Why We Should Care about Ultra-Rare Disease.” European Respiratory Review 25(140):101–3. doi: 10.1183/16000617.0017-2016.

Mattos, Samuel Miranda, Virna Ribeiro Feitosa Cestari, and Thereza Maria Magalhães Moreira. 2023. “Scoping protocol review: PRISMA-ScR guide refinement.” Rev Enferm UFPI 12(1). doi: 10.26694/reufpi.v12i1.3062.

Máximo, Carlos Henrique M. 2003. “Acesso percutâneo nas coleções e pseudocistos pancreáticos.” Radiologia Brasileira 36:168–168. doi: https://doi.org/10.1590/S0100-39842003000300015.

Ouzzani, Mourad, Hossam Hammady, Zbys Fedorowicz, and Ahmed Elmagarmid. 2016. “Rayyan—a Web and Mobile App for Systematic Reviews.” Systematic Reviews 5(1):210. doi: 10.1186/s13643-016-0384-4.

Peters, Micah D. J., Casey Marnie, Andrea C. Tricco, Danielle Pollock, Zachary Munn, Lyndsay Alexander, Patricia McInerney, Christina M. Godfrey, and Hanan Khalil. 2020. “Updated Methodological Guidance for the Conduct of Scoping Reviews.” JBI Evidence Synthesis 18(10):2119–26. doi: 10.11124/JBIES-20-00167.

Peters, Micah D.J.1,2,3; Godfrey, Christina; McInerney, Patricia; Khalil, Hanan; Larsen, Palle8; Marnie, Casey; Pollock, Danielle; Tricco, Andrea C.; Munn, Zachary. Best practice guidance and reporting items for the development of scoping review protocols. JBI Evidence Synthesis 20(4):p 953-968, April 2022. | DOI: 10.11124/JBIES-21-00242

Rivitti, E A. 2024. Manual de Dermatologia Clinica de Sampaio e Rivitti - Rivitti. - Livraria Florence. (2nd ed.). Grupo A.

Schroyens, A., B. Sykes, L. Kwok, C. Landgren, and L. O’Connell. 2012. Description of a rare and novel disease: tempi syndrome. 97, 608–608.

Smith, C. I. Edvard, Peter Bergman, and Daniel W. Hagey. 2022. “Estimating the Number of Diseases – the Concept of Rare, Ultra-Rare, and Hyper-Rare.” iScience 25(8), 104698. doi: 10.1016/j.isci.2022.104698.

Sykes, David B., Casey O’Connell, and Wilfried Schroyens. 2020. “The TEMPI Syndrome.” Blood 135(15), 1199–1203. doi: 10.1182/blood.2019004216.

Sykes DB, Schroyens W, and O’Connell C. 2011. The TEMPI Syndrome--a Novel Multisystem Disease. The New England Journal of Medicine 365(5), 475–77. doi: 10.1056/NEJMc1106670.

Tricco, Andrea C., Erin Lillie, Wasifa Zarin, Kelly K. O’Brien, Heather Colquhoun, Danielle Levac, David Moher, Micah D. J. Peters, Tanya Horsley, Laura Weeks, Susanne Hempel, Elie A. Akl, Christine Chang, Jessie McGowan, Lesley Stewart, Lisa Hartling, Adrian Aldcroft, Michael G. Wilson, Chantelle Garritty, Simon Lewin, Christina M. Godfrey, Marilyn T. Macdonald, Etienne V. Langlois, Karla Soares-Weiser, Jo Moriarty, Tammy Clifford, Özge Tunçalp, and Sharon E. Straus. 2018. “PRISMA Extension for Scoping Reviews (PRISMA-ScR): Checklist and Explanation.” Annals of Internal Medicine 169(7), 467–73. doi: 10.7326/M18-0850.

Xu, Jian, Wenqi Liu, Fengjuan Fan, Bo Zhang, Fei Zhao, Yu Hu, and Chunyan Sun. 2022. “TEMPI Syndrome: Update on Clinical Features, Management, and Pathogenesis.” Frontiers in Endocrinology 13, 886961. doi: 10.3389/fendo.2022.886961.

Downloads

Published

2025-03-13

Issue

Section

Note Preview

How to Cite

How scientific evidence can contribute to improve the diagnosis and comprehensive care of patients with TEMPI syndrome: A scoping review protocol. Research, Society and Development, [S. l.], v. 14, n. 3, p. e3614348388, 2025. DOI: 10.33448/rsd-v14i3.48388. Disponível em: https://ojs34.rsdjournal.org/index.php/rsd/article/view/48388. Acesso em: 28 jun. 2025.