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Special Article
367
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
Carmino Antonio de Souza
1
Katia Borgia Barbosa Pagnano
1
Israel Bendit
2
Monika Conchon
3
Carla Maria Boquimpani de Moura Freitas
4
Arthur Moellmann Coelho
5
Vaneuza Araújo Moreira Funke
6
Wanderley Marques Bernardo
2,7
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
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