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Overview
Chronic myeloid leukaemia is a rare cancer of the white blood cells and bone marrow. Although like many other cancers it can occur at any age, it is predominantly a disease of older adults with a median age of diagnosis ranging between 60 and 65 years (Baccarani et al, 2012). Incidence rates begin to rise from the mid-50 years upwards, with the highest rates in the 85–89 year group for males and over 90 years for females (Cancer Research UK, 2019). There are no specific statistics to clarify how likely a practice nurse is to encounter the disease, but there are approximately two new cases diagnosed each day in the UK (Cancer Research UK, 2019), and despite its rarity the disease accounts for approximately 15% of all leukaemia types (Granatovitz et al, 2015).
Pathophysiology
Chronic myeloid leukaemia occurs when something goes wrong in the genes of blood cells,
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Risk factors
Age is clearly a risk factor and, until recently, findings have produced mixed results on other predisposing risk factors. Exposure to organic solvents for a long duration (15–20 years) was found to be relevant, and similarly an occupational exposure to electro-magnetic fields may have an effect, although the evidence is weak (Bjork et al, 2001). In contrast, exposure to benzene, gasoline or diesel, and tobacco smoking were found to have no effect, while personal use of hair dye provided inconclusive data (Bjork et al, 2001).
A study by Strom et al (2009) has shown obesity to be a potential risk factor in chronic myeloid leukaemia. A high body mass index may be associated with a 2–3-fold increased risk of developing chronic myeloid leukaemia, and weight gain of more than 1 kg per year during early adulthood may be associated with a 4-fold increased risk (Strom et al, 2009).
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Symptoms
A patient presenting with any of the following symptoms would require an urgent full blood count within 45 hours:
- persistent fatigue
- unexplained bruising or bleeding
- unexplained fever
- enlarged spleen
- generalised lymphadenopathy and pallor (National institute for Health and Care Excellence (NICE), 2016)
More commonly, an abnormal blood test may be found on routine blood testing, and approximately 40% of those diagnosed with the disease are asymptomatic when they are initially diagnosed (Appleby et al, 2005).
The hallmark of diagnosis is leukocytosis with basophilia and immature granulocytes (Baccarani et al, 2012). Onset of the disease is slow and when symptoms eventually develop, they are usually mild and may easily be confused with more common diseases. Additional symptoms include loss of appetite, weight loss, headaches, sweating at night and bone pain (Cancer Research UK 2022). Other less common symptoms are shown in Table 1.
| Table 1. Rarer symptoms of chronic myeloid leukaemia |
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Diagnosis
Once the diagnosis is suspected, urgent referral is made, where further investigations will be initiated. A bone marrow biopsy will be arranged and samples are sent for analysis to look for the presence of leukaemia cells. In healthy individuals, the bone marrow sample comprises of blood forming cells and some fat cells. When the bone marrow has a much higher number of blood-forming cells than expected, it is hypercellular. In people with chronic myeloid leukaemia, the bone marrow is hypercellular because it is full of leukaemia cells (American Cancer Society, 2023). Samples are also analysed for the presence of the Philadelphia chromosome - the chromosome responsible for the BCR/ABL gene mutation.
Finding the Philadelphia chromosome is helpful in diagnosing the disease, and the presence of BCR/ABL rearrangement is regarded as the hallmark of chronic myeloid leukaemia, and is considered diagnostic when present in a patient with clinical manifestations of chronic
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Treatment
With treatment, the disease can often be controlled and patients may live a normal life. Treatment is guided by the phase of the disease and aims to alleviate any symptoms and delay progression whenever possible. Tyrosine kinase inhibitors have transformed the outcomes of patients with chronic myeloid leukaemia over the last 15 years and achieve their effect by interfering with the interaction between the BCR/ABL oncoprotein and adenosine triphosphate, thereby blocking proliferation of the malignant cells (Granatovicz et al, 2015). Common side effects of treatment are shown in Table 3.
| Table 3. Common side effects of treatment for chronic myeloid leukaemia | |
| Tyrosine kinase inhibitors | Chemotherapy |
| Headaches | Nausea |
| Skin rashes | Tiredness |
| Join pains | Loss of appetite |
| Fluid retention | Bruising and bleeding |
| Abnormal liver function tests | Weight loss |
| Fatigue | A lower resistance to infections |
| Diarrhoea | |
| Nausea | |
|
From: Cancer Research UK, 2019 |
|
Biological therapies
These drugs are usually given as
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NMC proficiencies
Nursing and Midwifery Council: standards of proficiency for registered nurses
Part 1: Procedures for assessing people’s needs for person-centred care
1. Use evidence-based, best practice approaches to take a history, observe, recognise and accurately assess people of all ages
Part 2: Procedures for the planning, provision and management of person-centred nursing care
3. Use evidence-based, best practice approaches for meeting needs for care and support with rest, sleep, comfort and the maintenance of dignity, accurately assessing the person’s capacity for independence and self-care and initiating appropriate interventions
11. Procedural competencies required for best practice, evidence-based medicines administration and optimisation
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Resources
American Cancer Society. Do we know what causes chronic myeloid leukaemia? 2023. www.cancer.org/cancer/chronic-myeloid-leukemia.html (accessed 30 November 2023)
American Society of Clinical Oncology. Leukemia - chronic myeloid – CML: types of treatment. 2018. https://www.cancer.net/cancer-types/leukemia-chronic-myeloid-cml/types-treatment (accessed 30 November 2023)
Appleby N, Burke E, Curran TA, Neary E. Chronic myeloid leukaemia: molecular abnormalities and treatment options. Trinity Student Medical Journal. 2005;6(1)
Baccarani M, Pileri S, Steegmann JL et al. Chronic myeloid leukemia: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up: On behalf of the ESMO Guidelines working group. Ann Oncol. 2012;23 Suppl 7:vii72–7. https://doi.org/10.1093/annonc/mds228
Besa E. Chronic myelogenous leukaemia. 2023. https://emedicine.medscape.com/article/199425-overview (accessed 30 November 2023)
Bjork J, Albin M, Welinder H, et al. Are occupational, hobby, or lifestyle exposures associated with Philadelphia chromosome positive chronic myeloid leukaemia? Occup Environ Med. 2001;58(11):722-727. https://doi.org/10.1136/oem.58.11.722
Cancer Research UK. Chronic myeloid leukaemia statistics. 2019. www.cancerresearchuk.org/health-professional/cancer-statistics/statistics-by-cancer-type/leukaemia-cml#heading-Zero (accessed 30 November 2023)
Cancer Research UK. Symptoms of chronic myeloid leukaemia. 2022.
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