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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 British Society for Haematology (BSH 2020), the European Society of Medical Oncology (ESMO 2017), and the American Academy of Family Physicians (AAFP 2014). ...
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Screening and diagnosis

Differential diagnosis: as per AAFP 2014 guidelines, take into consideration the common causes, such as infection, inflammation, and stress response, when evaluating patients with leukocytosis.
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Classification and risk stratification

Prognostic assessment: as per BSH 2020 guidelines, consider calculating the EUTOS Long-Term Survival score to inform prognosis.
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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

Specific circumstances

Pregnant patients: as per BSH 2020 guidelines, ensure that females with CML wishing to conceive do not have a previous TKI resistance and ideally have maintained MR4 for at least 12 months before considering discontinuation.
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Follow-up and surveillance

Surveillance for secondary malignancies: as per AAFP 2014 guidelines, obtain serial CBC monitoring and age- and sex-specific cancer screening since leukemia survivors are at increased risk of secondary malignancies.
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