chronic myeloid leukaemia

1. 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 platelets • Resulting in bruising

2. chronic myeloid leukaemia Chronic myeloid leukaemia (CML) is caused by…… • Radiation • Hiroshima • Nagasaki • Chernobyl (?) • Pylons (!) • Chemical • Benzene • Smoking • Obesity • 26% increased risk • Unknown • Most cases

3. chronic myeloid leukaemia 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 .

4. chronic myeloid leukaemia • 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

5. chronic myeloid leukaemia 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 abdomen Due to splenic enlargement Bleeding & Bruising Due to thrombocytopenia Infections Due to lack of functioning neutrophils

6. chronic myeloid leukaemia Lab findings: • Raised white cell count - with all sorts of myeloid precursors: • Blast cells • Promyelocytes • Myelocytes • Metamyelocytes • Anaemia • Thrombocytopenia

7. chronic myeloid leukaemia 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

8. chronic myeloid leukaemia Image from http://www.healthlinkbc.ca/kbase/media/medical/nci/cdr0000533336.jpg

9. chronic myeloid leukaemia • 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

10. chronic myeloid leukaemia • Treatment • Depends on the phase of the illness. • Chronic • Accelerated • Blast

11. chronic myeloid leukaemia • 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?

12. chronic myeloid leukaemia • 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.

13. chronic myeloid leukaemia 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 viscosity blood transfusions - for anaemia antibiotics – for infections can keep patients reasonably well for a long time.

14. chronic myeloid leukaemia 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.

15. chronic myeloid leukaemia 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.

16. chronic myeloid leukaemia 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

17. chronic myeloid leukaemia • 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

18. chronic myeloid leukaemia 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.

19. chronic myeloid leukaemia 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 !!)