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Chronic myeloid leukemia
Background
Overview
Definition
CML is a malignant clonal disorder of hematopoietic stem cells resulting in unregulated growth of myeloid cells in the bone marrow.
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Pathophysiology
CML is caused by a chromosomal abnormality termed as Philadelphia chromosome, which results from the presence of BCR-ABL oncogene.
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Disease course
Clinical manifestations include fatigue, weight loss, abdominal fullness, bleeding, purpura, splenomegaly, leukocytosis, anemia, and thrombocytosis. Disease progression may lead to bone pain, hepatosplenomegaly, severe anemia, infections, lymphadenopathy, CNS disease, and increased risk of death.
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Prognosis and risk of recurrence
CML is associated with a mortality crude rate of 0.78.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of chronic myeloid leukemia are prepared by our editorial team based on guidelines from the American Society for Transplantation and Cellular Therapy & Cell Therapy Transplant Canada (ASTCT 2024), the British Society for Haematology (BSH 2020), the European Society of Medical Oncology (ESMO 2017), and the American Academy of Family Physicians ...
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Screening and diagnosis
Classification and risk stratification
Diagnostic investigations
Diagnostic blood tests: as per ESMO 2017 guidelines, recognize that the diagnosis in most cases can be made on the basis of a characteristic blood count and differential (excessive granulocytosis with a typical left shift of granulopoiesis).
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Further evaluation
Diagnostic procedures
Bone marrow aspiration: as per BSH 2020 guidelines, obtain bone marrow aspiration at diagnosis for full karyotype analysis and to confirm the phase of the disease.
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Immunohistochemistry
Cytogenetic analysis
Mutational analysis
Medical management
Management of chronic phase, front-line regimens: as per BSH 2020 guidelines, offer imatinib as first-line therapy in most adult and pediatric patients with CML in chronic phase.
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Management of chronic phase (prevention of complications)
Management of chronic phase (treatment monitoring)
Management of chronic phase (treatment discontinuation)
Management of resistant/refractory disease
Management of accelerated/blast phase
Management of infection
Management of donor-specific anti-HLA antibodies (prevention)
Management of donor-specific anti-HLA antibodies (testing)
Management of donor-specific anti-HLA antibodies (desensitization therapy)
Management of donor-specific anti-HLA antibodies (follow-up)
Management of non-donor-specific anti-HLA antibodies