Chronic Myeloid Leukaemia Flashcards

1
Q

Define Chronic Myeloid Leukaemia (CML)?

A

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

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2
Q

What is the aetiology of CML?

A

Malignant proliferation of stem cells

95% of cases have a chromosomal translocation between chromosomes 9 and 22 to form the Philadelphia chromosome

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3
Q

What are some of the variants of CML?

A

Ph-negative CML
Chronic Neutrophilic Leukaemia
Eosinophilic Leukaemia

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4
Q

What is the pathogenesis of CML?

A

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

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5
Q

What are the three stages of CML?

A

Relatively stable chronic phase (4-6 yr duration)
Accelerated phase (3-9 months)
Acute Leukaemia phase (blast transformation)

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6
Q

What is the epidemiology of CML?

A

Incidence increases with age
Mean age of diagnosis: 40-60 yrs
4x more common in MALES

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7
Q

What are the presenting symptoms of CML?

A

Asymptomatic in 40-50% of cases - diagnosed in routine blood count
Hypermetabolic Symptoms
Bone Marrow Failure Symptoms

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8
Q

What are the Hypermetabolic Symptoms in CML?

A

Weight Loss
Malaise
Sweating

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9
Q

What are the Bone Marrow Failure symptoms in CML?

A
Lethargy
Dyspnoea 
Easy bruising 
Epistaxis 
Abdominal discomfort and early satiety
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10
Q

What are the rare symptoms of CML?

A
Gout 
Hyperviscosity symptoms (visual disturbance, headaches, pripaism)
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11
Q

What might happen in an blast crisis with CML?

A

May present with symptoms of AML and ALL

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12
Q

What are the signs of CML on physical examination?

A

Splenomegaly - most common physical finding (90% of cases)

Signs of Bone Marrow Failure (Pallor, Bleeding, Ecchymosis)

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13
Q

What bloods would you do for CML and what would you see?

A
High WCC
Low Hb 
High Basophils/neutrophils/eosinophils 
High/normal/low platelets 
High uric acid 
High B12 and transcobalamin I
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14
Q

What would you see on a blood film for CML?

A

Immature granulocytes

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15
Q

What would you see in a Bone Marrow Aspirate or Biopsy for CML?

A

Hypercellular with raised myeloid-erythoid ratio

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16
Q

What would you see if you did cytogenetics for CML?

A

Show the philadelphia chromosome