Chronic Myelogenous Leukemia Overview
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Questions and Answers

Which characteristic is specifically associated with Chronic Myelogenous Leukemia (CML)?

  • Hypereosinophilia
  • BCR-abl fusion gene (correct)
  • Loss of hematopoietic stem cells
  • Increased monocytosis

What percentage of patients are typically diagnosed in the chronic phase of CML?

  • 85% (correct)
  • 75%
  • 50%
  • 65%

What clinical symptom is NOT typically associated with Chronic Myelogenous Leukemia?

  • Epistaxis
  • Menorrhagia
  • Visual disturbances
  • Peripheral neuropathy (correct)

Which of the following is a common feature shared by chronic myeloproliferative disorders?

<p>Splenomegaly (D)</p> Signup and view all the answers

What is the male-to-female incidence ratio of Chronic Myelogenous Leukemia?

<p>More common in men (D)</p> Signup and view all the answers

Which of the following classifications of CML involves the Philadelphia chromosome?

<p>Classical CML with Philadelphia positive (A)</p> Signup and view all the answers

How is Chronic Myelogenous Leukemia classified based on cell precursors?

<p>Classified by the specific cell precursors involved (D)</p> Signup and view all the answers

What demographic is most commonly affected by Chronic Myelogenous Leukemia?

<p>Middle-aged and elderly (B)</p> Signup and view all the answers

What is a characteristic finding in the complete blood count (CBC) of a patient with chronic myeloid leukemia (CML)?

<p>Normocytic, normochromic anemia (D)</p> Signup and view all the answers

Which of the following is a phase of Chronic Myelogenous Leukemia?

<p>Chronic phase (D)</p> Signup and view all the answers

What would be an expected finding in the bone marrow of a patient with chronic myeloid leukemia?

<p>Increased myeloid to erythroid (M:E) ratio (B)</p> Signup and view all the answers

Which environmental factor is linked to an increased risk of developing CML?

<p>Long-term exposure to benzene (C)</p> Signup and view all the answers

Which of the following biochemical markers is expected to be increased in a patient with chronic myeloid leukemia?

<p>Both B and C (D)</p> Signup and view all the answers

Which immunological marker is positive in chronic myeloid leukemia?

<p>CD15 (D)</p> Signup and view all the answers

What type of blood cell count pattern is commonly seen in patients with CML?

<p>Elevated WBC count with moderate thrombocytosis (C)</p> Signup and view all the answers

What is the significance of the Philadelphia chromosome in chronic myeloid leukemia?

<p>It is associated with the BCR-ABL fusion gene. (C)</p> Signup and view all the answers

Which of the following findings would indicate the chronic phase of chronic myeloid leukemia?

<p>Blasts not more than 10% (B)</p> Signup and view all the answers

Which hematological exam could show the presence of immature myeloid precursors away from bony trabeculae?

<p>Bone marrow biopsy (C)</p> Signup and view all the answers

What is a common peripheral blood film feature in chronic myeloid leukemia?

<p>Neutrophils with left shift (B)</p> Signup and view all the answers

In chronic myeloid leukemia, which of the following is a likely cause of increased serum uric acid?

<p>Increased purine destruction (C)</p> Signup and view all the answers

What characterizes the accelerated phase of chronic myeloid leukemia (CML)?

<p>More than 20% basophils in the blood or bone marrow (D)</p> Signup and view all the answers

Which of the following is associated with the Philadelphia chromosome?

<p>It produces a specific 210kD tyrosine kinase (C)</p> Signup and view all the answers

Which laboratory test is NOT typically part of the diagnosis for chronic myeloid leukemia?

<p>Celiac disease panel (C)</p> Signup and view all the answers

What is a key effect of the enhanced tyrosine kinase activity seen in CML?

<p>Promotion of abnormal blood cell production (B)</p> Signup and view all the answers

What happens to red blood cell and platelet production in chronic myeloid leukemia?

<p>Production is insufficient due to replacement by blasts (A)</p> Signup and view all the answers

Which of the following phases is characterized by myeloid blast crises in CML?

<p>Acute phase with over 1000x blasts (C)</p> Signup and view all the answers

What is the significance of having a blast count higher than 20% in CML?

<p>Points towards an accelerated phase (C)</p> Signup and view all the answers

Which statement accurately describes the symptomatology of chronic myeloid leukemia during its chronic phase?

<p>Patients can be asymptomatic or have mild symptoms (B)</p> Signup and view all the answers

How do abnormal myeloid cells affect the body's immune response in CML?

<p>They cannot function properly, leading to increased infection risk (C)</p> Signup and view all the answers

What is a likely consequence of excessive proliferation of immature blood cells (blasts) in CML?

<p>Suppression of normal hematopoiesis (D)</p> Signup and view all the answers

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Flashcards

What is Chronic Myelogenous Leukemia (CML)?

A type of cancer that affects the bone marrow and causes too many white blood cells to be produced.

What is the defining characteristic of CML?

CML is a type of blood cancer that is characterized by an abnormal fusion gene called BCR-ABL.

What are myeloproliferative disorders?

A group of blood cancers that arise from abnormal stem cells in the bone marrow, leading to an overproduction of specific blood cells.

How is CML classified?

CML is classified based on the presence or absence of the Philadelphia chromosome, a genetic abnormality that can be identified in the cells.

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What is the chronic phase of CML?

The chronic phase is the initial stage of CML, when the disease is generally more manageable and less aggressive.

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What is the accelerated phase of CML?

The accelerated phase is a more aggressive stage of CML, with faster disease progression and increased risk of complications.

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What is the blast crisis phase of CML?

The blast crisis is the final and most aggressive stage of CML, characterized by a rapid increase in blast cells, which are immature white blood cells.

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Who is more likely to get CML?

CML is more common in middle-aged and elderly individuals and has a higher prevalence in men.

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What factors can increase the risk of developing CML?

Exposure to radiation, like the atomic bombs in Hiroshima and Nagasaki, or to certain chemicals, like benzene, can increase the risk of developing CML.

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What is splenomegaly in CML?

The characteristic enlargement of the spleen, a common symptom of CML.

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What are the phases of CML?

CML progresses through distinct phases, each characterized by different blast percentages, platelet count, and other clinical features.

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What defines the Chronic Phase of CML?

The chronic phase is the initial stage of CML, typically lasting for several years. It is characterized by a low percentage of blasts (<10%), no spleenomegaly or anemia, and high platelet count.

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What defines the Accelerated Phase of CML?

The accelerated phase marks a significant shift, with a higher percentage of blasts (10-19%), more basophils, lower platelet count, and potential for large blast clusters in the bone marrow.

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What defines the Blast Crisis of CML?

The blast crisis is the most severe and aggressive phase of CML, with extremely high blast percentages (>20%). This phase is often fatal without treatment.

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What is the Philadelphia chromosome?

Philadelphia chromosome is an abnormal chromosome created by a translocation between chromosomes 9 and 22. This translocation leads to the fusion of the ABL and BCR genes, resulting in the production of a tyrosine kinase.

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What is the role of BCR-ABL in CML?

The BCR-ABL fusion gene produces a tyrosine kinase (p210) that promotes uncontrolled cell growth and division. This leads to the overproduction of abnormal blood cells, causing the symptoms of CML.

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What is the pathophysiology of CML?

CML arises due to the abnormal growth and division of myeloid blood cells, leading to a buildup of immature cells (blasts) in the bone marrow. These blasts can't function properly, affecting normal blood cell production.

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How does CML affect blood cell production?

In CML, the overproduction of myeloid cells crowds out the production of normal blood cells like red blood cells and platelets, leading to anemia and an increased risk of infection.

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How does the BCR-ABL fusion protein affect cell function?

The BCR-ABL fusion protein increases the activity of tyrosine kinase in the cells. This enhances phosphorylation, which regulates metabolic pathways and acts as a receptor for growth factors. The increased activity leads to uncontrolled cell growth and may also inhibit apoptosis in hemopoietic cells.

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What are the laboratory diagnostic tools for CML?

Laboratory tests such as CBC (complete blood count), biochemical tests, bone marrow examination, cytogenetics, and immunological markers are used to diagnose and monitor CML.

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WBC Count in CML

The number of white blood cells (WBCs) in a sample of blood. In chronic myeloid leukemia (CML), the WBC count is often significantly elevated, ranging from 50 to 500 x109/l.

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Thrombocytosis in CML

An increase in the number of platelets in the blood. This is a common finding in CML, often related to increased production of platelets by the bone marrow.

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Peripheral Blood Film Examination

An examination of a blood sample under a microscope to assess the size, shape, and appearance of blood cells. In CML, the examination often reveals abnormalities in the white blood cells, including an increase in immature neutrophils and the presence of nucleated red blood cells.

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Hypercellular Bone Marrow in CML

A condition where the bone marrow is overactive, producing too many cells. This is a characteristic feature of CML, where the marrow is dominated by abnormal myeloid cells.

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Increased M:E Ratio in CML

A specific abnormality in the bone marrow where the ratio of myeloid cells to erythroid cells is significantly increased. Normally, this ratio is balanced, but in CML, it is greatly skewed towards myeloid cells.

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NAP Score in CML

A laboratory test that measures the activity of an enzyme called NADH diaphorase in neutrophils. In CML, the NAP score is often decreased, reflecting the abnormal function of the neutrophils.

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Philadelphia Chromosome (Ph) in CML

A genetic abnormality found in the chromosomes of some cells. In CML, a specific translocation known as the Philadelphia chromosome (Ph) is often detected. This translocation involves the fusion of two genes, BCR and ABL.

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Immunological Markers in CML

A group of proteins found on the surface of cells that can be used to identify different types of cells. In CML, the abnormal myeloid cells often express specific markers such as CD13, CD14, CD15, and CD33.

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BCR-ABL Fusion Protein

A laboratory test that helps to detect the presence of the BCR-ABL fusion protein. In CML, the BCR-ABL fusion protein is often found in high levels, confirming the diagnosis of the disease.

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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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CML: Chronic Myelogenous Leukemia PDF

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.

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