chronic myeloid leukaemia cancer of granulocyte production too many (non functioning) granulocytes...

chronic myeloid leukaemia

•Cancer of granulocyte production •Too many (non functioning) granulocytes are produced

•Bone marrow is overcrowded with ineffective white cells•Resulting in infections

•Bone marrow is overcrowded with white cells it cannot make enough healthy red cells•Resulting in anaemia

Bone marrow is overcrowded with white cells it cannot make enough healthy plateletsResulting in bruising


Chronic myeloid leukaemia (CML) is caused by……

Radiation

•Hiroshima•Nagasaki•Chernobyl (?)•Pylons (!)

Chemical

•Benzene•Smoking

Obesity

•26% increased risk

Unknown

•Most cases

.


Symptoms:

None…..

Because CML progresses slowly, it is difficult to detect in its early stages. Sometimes it is only discovered when a blood test is done for another reason


Symptoms:

The main signs and symptoms are due to

•increased number of abnormal white blood cells in the bone marrow

•causing reduced production of normal blood cells


Symptoms:

Pallor & lethargy, due to a lack of red blood cells (anaemia).(often the first symptom patients see their doctor about)

A feeling of fullness or a tender lump on the left side of the abdomenDue to splenic enlargement

Bleeding & Bruising Due to thrombocytopenia

InfectionsDue to lack of functioning neutrophils

Lab findings:


Raised white cell count - with all sorts of myeloid precursors:

•Blast cells•Promyelocytes•Myelocytes•Metamyelocytes

Anaemia

Thrombocytopenia

The Philadelphia chromosome

The Philadelphia chromosome develops during cell division

part of chromosome 9 (the ABL gene) wrongly attaches to chromosome 22 (the BCR gene).

This creates a new gene, known as BCR-ABL

which produces a specific new protein called p210

disturbing the production of blood cells by stem cells in the bone marrow



Image from http://www.healthlinkbc.ca/kbase/media/medical/nci/cdr0000533336.jpg


An aside

CML is different to CLL in that:

•CLL is a disease of the elderly•Which in many cases does not reduce life expectancy.•CML is a disease of (usually) middle age. (45 – 55)•CML after some years transforms to acute leukaemia


Treatment

Depends on the phase of the illness.

•Chronic•Accelerated•Blast


Treatment

Chronic phase

control the condition, often for several years, prevent / treat symptoms of the disease so that quality of life is good.

•interferon alpha•Chemotherapy•stem cell transplant?

Treatment - Interferon alpha

a protein normally produced by the body during viral infections.

Interferon alpha is given daily or three times a week as a small injection under the skin using a very fine needle. The injections can be done at home.

Interferon alpha can cause side effects similar to the symptoms of `flu,

•Chills• fever•headache •aching in the back, joints and muscles•Lethargy.



Treatment

Chronic phase - Supportive treatments

Other treatments may be given to deal conditions which occur as a result of CML.

leukopheresis - (using a cell separator) to reduce viscosityblood transfusions - for anaemiaantibiotics – for infections

can keep patients reasonably well for a long time.


Treatment

Accelerated phase

Treatment is usually a combination of chemotherapy drugs, given intravenously.

High-dose treatment with a stem cell transplant may also be used for some people.


Treatment

Blast phase

try to put the leukaemia back into a second chronic phase.

Because blast phase CML is like an acute leukaemia, combinations of chemotherapy drugs that are used to treat acute leukaemia are often used.

High-dose treatment with a stem cell transplant may also be an option if the leukaemia responds well to a combination of chemotherapy drugs.


Treatment - Bone marrow transplantation

Autologous

The idea behind this procedure is to allow people who do not have a donor to have the benefit of high dose chemotherapy. Autologous transplants use patient’s own stem cells.

Stem cells will be collected while free of any signs of the disease (in remission).

And are returned to the patient after high dose chemotherapy


Treatment - Bone marrow transplantation

Allogenic

An autologous transplant is merely replacing leukaemic cells with cells proven to go leukaemic

So in theory patients are better off with an allogenic transplant.

Provided

•it doesn’t get rejected•it doesn’t reject the patient


Treatment - In the future

researchers are trying to find a way to switch off the BCR-ABL gene

and so stop CML’s symptoms from developing.


Prognosis:

Untreated CML usually transforms to an acute phase some 4-5 years after presentation.

As treatment improves, cure rates are increasing.

(This statement is deliberately vague !!)

chronic myeloid leukaemia cancer of granulocyte production too many (non functioning) granulocytes...

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