Chronic Myeloid Leukaemia

Page 3: Learning Objectives

  • Review of haematopoiesis.

  • Identify clinical and laboratory characteristics of CML.

  • Discuss molecular biology of the Philadelphia (Ph) Chromosome.

  • Understand the mechanism of tyrosine kinase inhibitors (TKIs).

  • Examine treatment monitoring systems for CML.

Page 5: Haematopoiesis Overview

  • Prenatal and Postnatal Haematopoiesis:

    • Yolk sac (foetal) → Bone marrow (postnatal).

Page 6: Haematopoiesis Cell Types

  • Hematopoietic Stem Cells: Multipotent origin.

  • Differentiation pathways include:

    • Myeloid Progenitors: Erythrocytes, platelets.

    • Lymphoid Progenitors: T and B lymphocytes, natural killer cells.

  • Key cell types include myeloblast, lymphoblast, and various progenitor cells.

Page 7: Morphology of White Blood Cells

  • Overview and normal proportion of white blood cells:

    • Neutrophils (62%), Lymphocytes (30%), Monocytes (5%), Eosinophils (2.5%), Basophils (0.75%).

Page 9: Mutations in Haematopoiesis

  • Clonal haematopoiesis and its evolution through various factors: aging, inflammation, environmental impacts.

  • Specific mutations associated with hematologic neoplasms include DNMT3A, TET2.

Page 10: Classification of Blood Cancers

  • Liquid Tumours:

    • Leukaemia: Chronic, Acute.

    • Multiple Myeloma, Myelodysplasia.

  • Solid Tumours:

    • Hodgkin's and Non-Hodgkin's Lymphoma.

Page 11: Chronic Myeloid Leukaemia (CML) Characteristics

  • A clonal myeloproliferative disorder affecting myeloid cells.

  • Key features include high myelocyte percentage & neutrophils in bone marrow.

  • Stats: 15-20% of all leukaemia cases, more frequent in ages 65+, slight male predominance.

Page 13: CML Risk Factors

  • Involves ionizing radiation exposure, chemicals (e.g., benzene, cigarettes), immunodeficiency, also genetic predispositions based on age and sex.

Page 14: Overview of Earlier Studies on CML

  • Previous findings have linked environmental factors with increased risks of leukaemia, emphasizing the role of exposures to harmful substances.

Page 15: CML Incidence and Outcome Statistics

  • Approx.: 9,110 new cases in the UK per year, ~1,220 deaths.

  • Survival Rates: Variations based on age and gender.

Page 16: Common Symptoms of CML

  • Symptoms include unexplained fever, anaemia, headaches, frequent infections, and more.

Page 17: Detailed Clinical Symptoms of CML

  • Additional symptoms include night sweats, abdominal pain, and splenomegaly.

Page 18: CML Pathophysiology

  • First leukaemia linked to a genetic mutation (Philadelphia Chromosome, Ph).

  • 95% of CML patients harbour this translocation (9;22).

Page 20: Role of Tyrosine Kinases in CML

  • Define tyrosine kinases and their role in signaling pathways.

  • Mutations leading to sustained activity linked directly to cancer progression.

Page 21: Mechanism of Tyrosine Kinase Activation

  • Discusses ligand interactions and spontaneous dimerization.

Page 22: BCR-ABL Protein Structure

  • Diagram illustrating functions of BCR and ABL components.

Page 23: Diagnosis of CML

  • Initial evaluation includes blood tests, morphology, and cytogenetic analysis.

  • Critical for accurate classification and treatment planning.

Page 24: Detailed CML Diagnostic Criteria

  • Lab results typically show elevated WBC and low red blood cells and platelets.

Page 25: Additional Diagnostic Information

  • Further elaboration on diagnostic testing methods and clinical implications.

Page 26: Phases of CML

  • Characteristic durations and symptoms associated with each phase: Chronic, Accelerated, and Blast Crisis.

Page 27: Characteristics of Blast Crisis

  • Describes acute transformation indicating high blast percentages and treatment challenges.

Page 28: Future Treatments for CML

  • References to CRISPR and potential gene therapies being explored.

Page 29: Treatment Options for CML

  • Diverse strategies including targeted therapies, stem cell transplantation, chemotherapy, and supportive care.

Page 30: Details on Targeted Therapy for CML

  • Involves the use of tyrosine kinase inhibitors (TKIs) to manage cancer progression.

Page 31: Recent Studies on TKIs

  • References and studies examining combined therapies for improved CML outcomes.

Page 32: Specificity of Imatinib Efficacy

  • Imatinib hailed as groundbreaking therapy approved in 2001, maintaining BCR-ABL inhibition and delaying progression.

Page 33: Additional References on Imatinib Development

  • Continuation of discussions about targeting leukaemia therapies.

Page 34: Resource Reference for CML Explanation

  • Cited reference sources offer foundational knowledge in hematology.

Page 35: Continuation of Essential References for CML

  • Further bibliographic material relevant for understanding CML.

Page 36: Chemotherapy Overview for CML

  • Mechanisms of drugs utilized and their induction of apoptosis in leukemic cells.

Page 37: Clinical Trials for CML Treatments

  • Ongoing investigations include combinations of TKIs and traditional chemotherapy.

Page 38: Chemotherapy Side Effects

  • Notable toxicities associated with CML treatments impacting various organ systems.

Page 39: Multi-Drug Resistance (MDR) in CML

  • Definition and implications of MDR on treatment efficacy.

Page 40: Mechanisms Underlying MDR in CML

  • Detailed discussion on genetic factors influencing drug resistance.

Page 41: Stem Cell Transplantation for CML

  • Highlighted as a curative option for patients resistant to TKIs.

Page 42: Graft Versus Leukaemia Effect in CML

  • Importance of immune response post-transplant in achieving remission.

Page 43: Challenges in Stem Cell Transplantation

  • Discusses complications such as graft rejection and infections.

Page 44: Graft Versus Host Disease (GvHD)

  • Summary of GvHD implications post-transplant and related management strategies.

Page 45: Active vs Inactive GvHD Indications

  • Visual demonstration of active and inactive symptoms.

Page 46: Pre-Transplant Considerations for CML

  • Description of pertinent issues before eligibility for stem cell transplantation.

Page 47: Supportive Therapy for CML Patients

  • Therapeutic measures aimed at alleviating symptoms and side effects.

Page 48: Treatment Monitoring and Adjustments

  • Highlights how treatment effectiveness is assessed through various methodologies.

Page 49: Definitions of CML Treatment Responses

  • Explanation of response criteria as it relates to TKI therapy over time.

Page 50: Suggested Further Readings on CML

  • Recommendations for in-depth studies concerning CML advancements.

Page 51: Additional References for CML Studies

  • Extended literature for comprehensive understanding of CML dynamics.

Page 52: Conclusion and Feedback Section

  • Invitation for feedback on lecture material, possible improvements noted.