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Questions and Answers
Which characteristic is specifically associated with Chronic Myelogenous Leukemia (CML)?
Which characteristic is specifically associated with Chronic Myelogenous Leukemia (CML)?
What percentage of patients are typically diagnosed in the chronic phase of CML?
What percentage of patients are typically diagnosed in the chronic phase of CML?
What clinical symptom is NOT typically associated with Chronic Myelogenous Leukemia?
What clinical symptom is NOT typically associated with Chronic Myelogenous Leukemia?
Which of the following is a common feature shared by chronic myeloproliferative disorders?
Which of the following is a common feature shared by chronic myeloproliferative disorders?
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What is the male-to-female incidence ratio of Chronic Myelogenous Leukemia?
What is the male-to-female incidence ratio of Chronic Myelogenous Leukemia?
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Which of the following classifications of CML involves the Philadelphia chromosome?
Which of the following classifications of CML involves the Philadelphia chromosome?
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How is Chronic Myelogenous Leukemia classified based on cell precursors?
How is Chronic Myelogenous Leukemia classified based on cell precursors?
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What demographic is most commonly affected by Chronic Myelogenous Leukemia?
What demographic is most commonly affected by Chronic Myelogenous Leukemia?
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What is a characteristic finding in the complete blood count (CBC) of a patient with chronic myeloid leukemia (CML)?
What is a characteristic finding in the complete blood count (CBC) of a patient with chronic myeloid leukemia (CML)?
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Which of the following is a phase of Chronic Myelogenous Leukemia?
Which of the following is a phase of Chronic Myelogenous Leukemia?
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What would be an expected finding in the bone marrow of a patient with chronic myeloid leukemia?
What would be an expected finding in the bone marrow of a patient with chronic myeloid leukemia?
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Which environmental factor is linked to an increased risk of developing CML?
Which environmental factor is linked to an increased risk of developing CML?
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Which of the following biochemical markers is expected to be increased in a patient with chronic myeloid leukemia?
Which of the following biochemical markers is expected to be increased in a patient with chronic myeloid leukemia?
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Which immunological marker is positive in chronic myeloid leukemia?
Which immunological marker is positive in chronic myeloid leukemia?
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What type of blood cell count pattern is commonly seen in patients with CML?
What type of blood cell count pattern is commonly seen in patients with CML?
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What is the significance of the Philadelphia chromosome in chronic myeloid leukemia?
What is the significance of the Philadelphia chromosome in chronic myeloid leukemia?
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Which of the following findings would indicate the chronic phase of chronic myeloid leukemia?
Which of the following findings would indicate the chronic phase of chronic myeloid leukemia?
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Which hematological exam could show the presence of immature myeloid precursors away from bony trabeculae?
Which hematological exam could show the presence of immature myeloid precursors away from bony trabeculae?
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What is a common peripheral blood film feature in chronic myeloid leukemia?
What is a common peripheral blood film feature in chronic myeloid leukemia?
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In chronic myeloid leukemia, which of the following is a likely cause of increased serum uric acid?
In chronic myeloid leukemia, which of the following is a likely cause of increased serum uric acid?
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What characterizes the accelerated phase of chronic myeloid leukemia (CML)?
What characterizes the accelerated phase of chronic myeloid leukemia (CML)?
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Which of the following is associated with the Philadelphia chromosome?
Which of the following is associated with the Philadelphia chromosome?
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Which laboratory test is NOT typically part of the diagnosis for chronic myeloid leukemia?
Which laboratory test is NOT typically part of the diagnosis for chronic myeloid leukemia?
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What is a key effect of the enhanced tyrosine kinase activity seen in CML?
What is a key effect of the enhanced tyrosine kinase activity seen in CML?
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What happens to red blood cell and platelet production in chronic myeloid leukemia?
What happens to red blood cell and platelet production in chronic myeloid leukemia?
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Which of the following phases is characterized by myeloid blast crises in CML?
Which of the following phases is characterized by myeloid blast crises in CML?
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What is the significance of having a blast count higher than 20% in CML?
What is the significance of having a blast count higher than 20% in CML?
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Which statement accurately describes the symptomatology of chronic myeloid leukemia during its chronic phase?
Which statement accurately describes the symptomatology of chronic myeloid leukemia during its chronic phase?
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How do abnormal myeloid cells affect the body's immune response in CML?
How do abnormal myeloid cells affect the body's immune response in CML?
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What is a likely consequence of excessive proliferation of immature blood cells (blasts) in CML?
What is a likely consequence of excessive proliferation of immature blood cells (blasts) in CML?
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Study Notes
Chronic Myelogenous Leukemia (CML)
- CML is a clonal myeloproliferative disorder
- Characterized by a specific genetic abnormality: BCR-abl fusion gene
- Associated with leukocytosis (increased white blood cells) with a complete left shift
- Also shows basophilia (increased basophils) and splenomegaly (enlarged spleen) and hepatomegaly (enlarged liver)
- A myeloproliferative disorder is a group of disorders that are considered clonal malignancies of the hematopoietic stem cells.
- Other common features of myeloproliferative disorders include splenomegaly, hepatomegaly, increased leukocytosis (increased white blood cells), thrombocytosis (increased platelets), and erythrocytosis (increased red blood cells).
- Types of myeloproliferative disorders include chronic myelogenous leukemia (CML), myelofibrosis with myeloid metaplasia (MMM), polycythemia vera (PV), and essential thrombocythemia (ET).
- Synonyms for CML include chronic granulocytic leukemia, chronic myelocytic leukemia
Classification
- Based on the presence or absence of Philadelphia chromosome and the cell precursors involved.
- Includes classical CML with Philadelphia chromosome
- CML without Philadelphia chromosome
- CML of neutrophilic cell
- CML of eosinophilic cell
- CML of myelomonocyte
- Juvenile CML
Epidemiology
- CML affects all age groups but most common in middle-aged and elderly
- Incidence is 1-2 per 100,000 people
- More common in men
- Represents 15-20% of adult leukemia cases in Western populations
- Increased cases in populations exposed to atomic bombings (Hiroshima & Nagasaki)
- Long-term benzene exposure may contribute
Clinical Features
- Splenomegaly
- Gout-like symptoms
- Anemia
- Hyperuricemia
- Bruising
- Epistaxis
- Menorrhagia
- Hemorrhages from other sites
- Visual disturbance
Clinical Presentation
- CML is a malignant blood disorder
- Involves early hematopoietic cells that become clonally expanded
- Originates from a single abnormal hematopoietic stem cell which proliferates over time, resulting in blood granulocytosis and marrow granulocytopoiesis
- Marked by bone marrow hypercellularity
Phases of CML
- Chronic phase
- Accelerated phase
- Blast crises
Chronic Phase
- Approximately 85% of patients are in the chronic phase at diagnosis.
- Mostly asymptomatic or have mild symptoms.
- Blast cells are <10%.
- No splenomegaly
- No anemia
- Thrombocytosis
- Variable duration
- May progress to accelerated phase
Accelerated Phase
- 10-19% blasts in blood or bone marrow
-
20% basophils in blood or bone marrow
- Platelet count <100,000 which is unrelated to treatment.
- Other chromosomal abnormalities may be present
- Marked splenomegaly and increasing white blood cell count which is unresponsive to therapy
Blast Crises
- Final phase of CML evolution
- Resembles acute leukemia
- Rapid progression and short survival
- Diagnosis based on presence of:
-
20% myeloblasts or lymphoblasts in blood or bone marrow
- Large clusters of blasts in bone marrow biopsy
- Development of chloroma (solid focus of leukemia outside bone marrow)
-
Pathophysiology
- Every cell contains 23 pairs of chromosomes
- The most important cause is the translocation of two chromosomes:
- Chromosome 9 (ABL gene)
- Chromosome 22 (BCR gene)
- This translocation creates the Philadelphia chromosome (BCR-ABL)
- This new gene produces a specific 210kD tyrosine kinase
- This tyrosine kinase stimulates uncontrolled production of abnormal blood cells
General Details of CML
- Normally, white blood cells grow and divide in a controlled manner
- In CML, the process gets out of control, causing rapid division without proper maturation.
- Too many myeloid cells are produced, releasing into the bloodstream.
- Immature myeloid cells are called blasts and cannot function properly.
- Blast cells fill bone marrow and block normal blood cell production.
- This impairment leads to an increased risk of infection.
Lab Diagnosis
- Biochemical tests
- Bone marrow examination
- Immunological markers
- Cytogenetics
- Molecular assays
Lab Investigation of CML
- Complete blood count (CBC): Increased white blood cell count, and platelet count
- Blood cell count- WBC range from 50-500 x109/L
- Peripheral blood film examination:
- Normocytic, normochromic anaemia
- Neutrophils showing a left shift
- Eosinophils normal or sometime increased
- NRBCs are seen
- Absolute Basophilia
- Moderate thrombocytosis
- Macrocytosis
- Hypogranulated myeloid cells
Biochemical Findings
- Increased serum uric acid (due to increased purine destruction)
- Increased serum iron
- Increased serum B12 and B12 binding capacity
- Decreased NAP score
- Elevated serum LDH
- Elevated Ca++
Immunological Markers
- CD13 +
- CD14 +
- CD15 +
- CD33 +
Specialized Techniques
- Chromosome analysis: identifying Philadelphia chromosome (Ph+) and BCR-ABL positivity.
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Description
This quiz provides an overview of Chronic Myelogenous Leukemia (CML), a clonal myeloproliferative disorder characterized by the BCR-abl fusion gene. Explore its symptoms, including leukocytosis, basophilia, and splenomegaly, as well as other related myeloproliferative disorders. Test your knowledge on this vital hematological condition.
