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Chronic Myeloid Leukaemia

Title                    

Ñ    Chronic Myeloid Leukaemia

Definition

Ñ    Disorder of the multipotent stem cell

Ñ    Gross expansion of:

Myeloid progenitor cells

Subsequently mature granulocytic cell compartment

Pathogenesis

Ñ    The Philadelphia (Ph) chromosome t (9; 22) is formed by translocation of part of the long arm (q) of chromosome 22 to chromosome 9

Ñ    This creates a “fusion” gene, which is translated to produce a fusion protein which causes altered cell growth, stromal attachment and apoptosis

Results in:

Ñ    Increased cellularity of the bone marrow

Ñ    Mainly maturing granulocyte precursors

Clinical Features

History

Ñ    Chronic phase 3-4 years, which is followed by an accelerated phase, which may transform into:

Blastic transformation (80% myeloid 20 %`lymphoid)

Myelofibrosis

Age

Ñ    40-60 years

E&M

Ñ    Fever

Ñ    Night sweats

Ñ    Weight loss

RAG

Ñ    Priapism:

Leucostasis

HS

Ñ    Anaemia

Ñ    Abdominal discomfort:

Splenic enlargement

CNS

Ñ    Headaches:

Leucostasis

Ñ    Blurred vision:

Leucostasis

Examination

HS

Ñ    Anaemia

Ñ    Splenomegaly

CNS

Ñ    Retinal haemorrhages:

Leucostasis

Investigations

Fluids

Blood

Haematology

Ñ    Hb:

Normal or low

Ñ    White Cell Count:

Raised with the whole spectrum of myeloid precursors including a few blast cells

Ñ    Platelets:

Normal, low, raised

Ñ    Leucocyte alkaline phosphatase score:

Low

Genetics

Ñ    Cytogenetic analysis:

Ph chromosome is present in most patients

Ñ    RT-PCR (reverse transcriptase polymerase chain reaction):

Expression of BCR-ABL oncogene

Tissue Diagnosis

Bone marrow aspirate

Ñ    Hypercellular marrow with increase in myeloid precursors

Management 

Control

Drugs

Ñ    Imatinib (tyrosine kinase inhibitor):

Effective in inducing Philadelphia negative haematopoiesis

Ñ    Busulphan

Ñ    Hydroxyurea

Biological Agents

Ñ    Alpha interferon

Ñ    Allogenic bone marrow transplantation following myoablative therapy:

Limitations are donor availability, age, morbidity and mortality of procedure

Symptom relief

Ñ    Allopurinol:

To prevent secondary gout

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