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Chronic Myeloid Leukemia (Detailed)

Myeloproliferative neoplasm characterized by Philadelphia chromosome (BCR::ABL1 fusion) producing constitutively active tyrosine kinase, presenting in chronic phase with leukocytosis and splenomegaly, treated with tyrosine kinase inhibitors (imatinib, dasatinib, nilotinib, bosutinib, ponatinib, asciminib) achieving deep molecular response and near-normal life expectancy.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

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This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Chronic Myeloid Leukemia (Detailed)?

Chronic myeloid leukemia (CML) is a clonal myeloproliferative neoplasm characterized by reciprocal translocation t(9;22)(q34;q11) creating the Philadelphia chromosome and BCR::ABL1 fusion oncogene that produces a constitutively active tyrosine kinase driving uncontrolled myeloid proliferation. Annual incidence is 1-2 per 100,000, with median age 64 years. CML progresses through three phases: chronic phase (>=85% of cases at diagnosis, indolent course with mature granulocyte expansion), accelerated phase (10-19% blasts, increased basophils, cytogenetic clonal evolution), and blast phase (>=20% blasts, behaves like acute leukemia, poor prognosis).

Diagnosis requires confirmation of BCR::ABL1 by reverse transcriptase PCR (quantitative for monitoring), fluorescence in situ hybridization (FISH), or cytogenetics showing t(9;22). Initial presentation is often incidental leukocytosis with left shift, mild anemia, thrombocytosis, and palpable splenomegaly. Symptoms when present include fatigue, weight loss, night sweats, abdominal fullness, and early satiety. Risk stratification uses Sokal, Hasford (Euro), or EUTOS-LTS scoring systems incorporating age, spleen size, blast percentage, basophils, eosinophils, and platelet count.

Tyrosine kinase inhibitors (TKIs) are the cornerstone of treatment, achieving complete cytogenetic response in >70% and deep molecular response (BCR::ABL1 IS <=0.01%) in 30-50% of patients. First-generation imatinib 400 mg daily is standard initial therapy with proven 10-year overall survival ~85%. Second-generation TKIs (dasatinib 100 mg daily, nilotinib 300 mg twice daily, bosutinib 400 mg daily) achieve faster and deeper molecular responses but with distinct toxicity profiles (dasatinib: pleural effusions, pulmonary hypertension; nilotinib: cardiovascular events, hyperglycemia; bosutinib: gastrointestinal toxicity). Third-generation ponatinib 45 mg daily targets T315I gatekeeper mutation but causes arterial thrombotic events. Asciminib (STAMP inhibitor) binds the myristoyl pocket allosterically, effective in resistance and approved for T315I and >=2 prior TKIs. Treatment-free remission can be attempted in patients with stable deep molecular response (MR4 or MR4.5) for >=2 years on TKI; approximately 50% achieve durable treatment-free remission with monitoring.

Symptoms

Fatigue and weight loss
Abdominal fullness and early satiety from splenomegaly
Night sweats and low-grade fever
Bone pain (especially sternum)
Easy bruising or bleeding
Pallor from anemia
Incidental leukocytosis on routine CBC
Splenomegaly on physical examination
Symptoms of accelerated/blast phase: high fever, severe bleeding, infection

Risk Factors

Age over 60 years (median diagnosis age 64)
Ionizing radiation exposure
Male sex (slightly more common)
No clear environmental or familial predisposition for most cases
Atomic bomb survivors (historical)
Prior radiotherapy

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent unexplained leukocytosis
  • Splenomegaly on examination
  • Fatigue with abnormal CBC
  • Bone pain with elevated white count
  • Easy bruising with platelet abnormalities
  • Incidental finding of left-shifted granulocytes
  • Family history of myeloproliferative neoplasm
  • Failure of response or progression on TKI therapy

Treatment Methods

01
Confirmation of BCR::ABL1 by RT-PCR, FISH, or cytogenetics
02
Risk stratification with Sokal, Hasford, or EUTOS-LTS scoring
03
First-line imatinib 400 mg daily or second-generation TKI
04
Second-generation TKIs (dasatinib, nilotinib, bosutinib) for higher-risk disease
05
Ponatinib for T315I mutation or multi-TKI resistance
06
Asciminib (STAMP inhibitor) for resistant disease
07
Quarterly molecular monitoring with quantitative BCR::ABL1 PCR
08
Treatment-free remission attempt in stable deep molecular responders
09
Allogeneic stem cell transplantation for advanced phase or TKI-resistant disease
10
Hydroxyurea for cytoreduction in newly diagnosed patients before BCR::ABL1 confirmation

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Hematoloji Department

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You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.