Chronic Myeloid Leukaemia Flashcards
Define Chronic Myeloid Leukaemia (CML)?
Chronic myeloblastic leukaemia is a malignant clonal disease characterised by proliferation of granulocyte precursors in the bone marrow and blood, distinguished from AML by its slower progression
What is the aetiology of CML?
Malignant proliferation of stem cells
95% of cases have a chromosomal translocation between chromosomes 9 and 22 to form the Philadelphia chromosome
What are some of the variants of CML?
Ph-negative CML
Chronic Neutrophilic Leukaemia
Eosinophilic Leukaemia
What is the pathogenesis of CML?
The philadelphia chromosome results in the formation of the BCR-ABL fusion gene
The product of this gene enhances tyrosine kinase activity and drives cell replication
What are the three stages of CML?
Relatively stable chronic phase (4-6 yr duration)
Accelerated phase (3-9 months)
Acute Leukaemia phase (blast transformation)
What is the epidemiology of CML?
Incidence increases with age
Mean age of diagnosis: 40-60 yrs
4x more common in MALES
What are the presenting symptoms of CML?
Asymptomatic in 40-50% of cases - diagnosed in routine blood count
Hypermetabolic Symptoms
Bone Marrow Failure Symptoms
What are the Hypermetabolic Symptoms in CML?
Weight Loss
Malaise
Sweating
What are the Bone Marrow Failure symptoms in CML?
Lethargy Dyspnoea Easy bruising Epistaxis Abdominal discomfort and early satiety
What are the rare symptoms of CML?
Gout Hyperviscosity symptoms (visual disturbance, headaches, pripaism)
What might happen in an blast crisis with CML?
May present with symptoms of AML and ALL
What are the signs of CML on physical examination?
Splenomegaly - most common physical finding (90% of cases)
Signs of Bone Marrow Failure (Pallor, Bleeding, Ecchymosis)
What bloods would you do for CML and what would you see?
High WCC Low Hb High Basophils/neutrophils/eosinophils High/normal/low platelets High uric acid High B12 and transcobalamin I
What would you see on a blood film for CML?
Immature granulocytes
What would you see in a Bone Marrow Aspirate or Biopsy for CML?
Hypercellular with raised myeloid-erythoid ratio