Special Article Carmino Antonio de Souza1 Katia Borgia Barbosa Pagnano1 Israel Bendit2 Monika Conchon3 Carla Maria Boquimpani de Moura Freitas4 Arthur Moellmann Coelho5 Vaneuza Araújo Moreira Funke6 Wanderley Marques Bernardo2,7 1 Universidade Estadual de Campinas - UNICAMP, Campinas, SP, Brazil 2 Faculdade de Medicina da Universidade de São Paulo - USP, São Paulo, SP, Brazil 3 Hospital Santa Marcelina – HSM, São Paulo, SP, Brazil 4 Hemocentro do Rio de Janeiro - HEMORIO, Rio de Janeiro, RJ, Brazil 5 Instituto Nacional do Câncer - INCA, Rio de Janeiro, RJ, Brazil 6 Universidade Federal do Paraná – UFPR, Curitiba, PR, Brazil 7 Associação Médica Brasileira - AMB, São Paulo, SP, Brazil Conflict-of-interest disclosure: The authors declare no competing financial interest Submitted: 12/06/2012 Accepted: 13/07/2012 Corresponding author: Carmino Antonio de Souza Centro de Hematologia e Hemoterapia de Campinas Universidade Estadual de Campinas- Unicamp. Rua Carlos Chagas, 450 – Cidade Universitária “Prof. Zeferino Vaz” 13083-878 Distrito de Barão Geraldo – Campinas, SP – Brazil. Phone: 55 19-3521 8740 carmino@unicamp.br www.rbhh.org or www.scielo.br/rbhh DOI: 10.5581/1516-8484.20120094 Rev Bras Hematol Hemoter. 2012;34(5):367-82 Chronic myeloid leukemia treatment guidelines: Brazilian Association of Hematology, Hemotherapy and Cell Therapy. Brazilian Medical Association Guidelines Project – 2012 Introduction The guidelines project is a joint initiative of the Associação Médica Brasileira and the Conselho Federal de Medicina. It aims to bring together information in medicine to standardize conduct in order to help decision-making during treatment. The data contained in this manuscript were prepared by and are recommended by the Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular. Even so, all possible conducts should be evaluated by the physician responsible for treatment depending on the patient’s setting and clinical status. Description of the method used to gather evidence: These guidelines were drafted after constructing 19 questions relevant to the diagnosis and treatment of Chronic myeloid leukemia (CML). The questions were structured using the PICO (patient-intervention-comparison-outcome) methodology, thus enabling the creation of strategies to search for evidence (Appendix 1) in the main scientific electronic databases (Medline/PubMed, Embase, Lilacs/SciELO, Cochrane Library, PreMEDLINE via OVID). Moreover a manual search for evidence in dissertations and theses was carried out (Biblioteca Digital de Teses e Dissertações do Instituto Brasileiro de Informação em Ciência e Tecnologia – BDTD/IBICT). Evidence was selected by critical evaluation using discriminatory instruments (scores) according to the category of question: diagnosis (Quality in Diagnostic and Screening tests - QADAS) or therapy (JADAD for randomized clinical trials and Newcastle-Ottawa scale for non-randomized studies). After identifying potential studies to substantiate recommendations, the level of evidence and degree of recommendation were calculated using the classification of Oxford (available at www.cebm.net). Summary of the degree of recommendation and level of evidence: A: Major experimental and observational studies. B: Minor experimental and observational studies. C: Case reports (non-controlled studies). D: Opinion without critical evaluation based on consensus, physiological studies or animal models. Aims To set parameters for clinical diagnosis, evaluate severity and standardize treatment, maintenance and monitoring options for CML patients. The target audience of these guidelines is the hematologist with the aim of contributing to decision making in the diagnosis and treatment of CML. What are the diagnostic criteria for Chronic myeloid leukemia? The diagnosis of CML is based on leukocytosis and often also thrombocytosis, and on the differential blood count (immature granulocytes, metamyelocytes, myeloblasts and basophilia). Diagnosis depends on the identification of the Philadelphia chromosome (22q) resulting from the t(9;22)(q34;q11) resulting in the head to tail fusion of Breakpoint Cluster Region (BCR) and the Abelson Murine Leukemia (AML) genes or identification of the result of this translocation in peripheral blood or bone marrow cells. In some cases, the Philadelphia chromosome cannot be detected and diagnosis is made by molecular methods. The typical clinical course has three stages: the chronic phase, the accelerated phase and the blast crisis phase. Most diagnoses are made in the chronic phase. The accelerated phase is defined as 367