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Leukaemia
This is an abnormal proliferation and expansion of white blood cells. Some leukaemias progress rapidly with patients often becoming very unwell over a short period of time. These are typically the ‘acute leukaemias’ and result in a failure of the normal bone marrow to work properly. Acute Leukaemia affects all age groups, but in childhood it is more commonly acute lymphoblastic leukaemia, while in adulthood, acute myeloid leukaemia is more common.
The chronic leukaemias tend to present in older adults, and often patients are diagnosed by chance when they have a full blood count test performed for another reason. Sometimes patients with chronic leukaemia do not need to start treatment when the diagnosis is made.
The acute leukaemias are aggressive cancers of the white blood cells. They can be divided into two main types, acute myeloid leukaemia (or AML) which affects around 2500 people in the UK each year and acute lymphoblastic leukaemia (or ALL) which affects 600-700 per year. Acute leukaemia is often thought of as a disease which affects children and indeed ALL is the commonest cancer affecting children however both diseases occur more commonly as people get older.
Typically acute leukaemia presents because of a failure of the bone marrow and failure of blood production. This may be with symptoms of anaemia such as fatigue, shortness of breath or just looking pale. A low platelet count may cause easy bruising or bleeding. In particular people may notice a fine pinprick type rash due to tiny bleeds in the skin. It can also cause excessive gum bleeding or nose bleeds. The white blood count may be low or high at the time of diagnosis but there are usually few effective white blood cells so infections such as a sore throat or chest infections are frequent and may be more serious than normal.
The diagnosis of leukaemia is not usually suspected when people first develop symptoms and most patients will present after a blood test and an urgent phone call from their GP or local haematology department. A bone marrow biopsy will be required to confirm the diagnosis and to provide important information about the genetics and molecular basis of the disease.
The outcome and treatment of acute myeloid leukaemia is dependent on this genetic information along with the assessment of other risk factors such as age, the level of the white count and whether the disease has developed secondary to previous chemotherapy or preexisting myelodysplasia. The response to the initial course of treatment is also important. Based on this the leukaemia is stratified into good, intermediate or high risk disease.
The treatment of acute myeloid leukaemia involves relatively intensive chemotherapy delivered over 8-10 days as an inpatient. This is followed by a period of very low blood
This is the commonest form of leukaemia in adults caused by an over growth of a type of white blood cell called B-lymphocytes in the blood. It is commonly referred to by its abbreviation ‘CLL’. Typically patients are more elderly, although we do have some much younger patients in our clinic. There is no clear cause for the disease, but it might start out as an unusual reaction to a low-key infection or auto-immune reaction. As the blood lymphocyte numbers increase, many patients remain entirely well. Indeed, the finding of CLL is often completely unexpected to the patient and GP alike. Patients typically have blood tests for other reasons such as diabetic or general health tests, and the elevated lymphocyte count is found by chance. Some patients, however, do present with more symptoms, such as worsening tiredness, weight loss, fevers or night sweats and some CLL patients also develop prominent swollen lymph glands, which may be in the neck, arm pits or groin. The spleen or liver might enlarge, and on occasion, a patient might notice some abdominal symptoms from these enlarged organs. Unfortunately CLL patients also have a tendency to pick up infections of their chest and sinuses as their normal immune system works less efficiently.
Patients with early stage CLL who have minimal symptoms are not usually treated, but observed on what is termed a ‘watch and wait’ programme. This usually means keeping an eye on various blood tests and the patient’s clinical state with visits 2 or 3 times a year. It can seem rather unexpected to patients to be diagnosed with a leukaemia but then not start treatment immediately, and this usually requires quite a bit of discussion and time to come to terms with. There are a number of reasons for not treating immediately. Quite a few patients can go many years before they actually start to develop significant symptoms from their leukaemia, and there has never been any evidence to show that early treatment makes patients live any longer.
When treatment is required, most standard treatments currently combine antibody therapy, most typically rituximab, with chemotherapy. There are a number of different chemotherapy choices, ranging from the more gentle, such as chlorambucil, to the more intensive, fludarabine plus cyclophosphamide. We go through the different options with each individual patient to make sure we get the right treatment for each patient. CLL treatments are almost always given as an outpatient, typically requiring one or two trips up to the Nuffield every month. For most patients, CLL responds very well to treatment and patients can enjoy remission that can last years. Usually the quality of life is good in this period, but unfortunately, CLL will inevitably get more active again, and further treatment will be required. There are, however, a number of very exciting new treatments that we hope will have licenses for relapsed disease in the near future.
On occasion we do consider much more intensive treatments such as stem cell transplantation for specific patients. This approach is potentially the only way to actually cure CLL, but is a much more serious undertaking than standard chemotherapy. We always explore this option where it may be appropriate.
These are uncommon bone marrow conditions where the bone marrow stem cells become overactive and make too many components of the blood. Typically patients might produce too many red cells, platelets or white cells. Some patients are diagnosed by chance when a blood count test is performed for other reasons, and the abnormal values in their blood come to light. Other patients may present with conditions such as blood clots, stroke, abnormal bleeding or infections. When there are too many red cells, the condition is known as Polycythaemia Rubra Vera, too many platelets typically means essential thromobocythaemia, and too many granulocyte white cells may be due to chronic myelomonocytic leukaemia, or chronic myeloid leukaemia. There is another related myeloproliferative disorder caused by an overgrowth of fibrotic tissue within the bone marrow. This is termed idiopathic myelofibrosis.
To investigate the above conditions, and help guide treatment decisions, the majority of patients will require a bone marrow biopsy as part of their initial investigations. We also arrange for specialist molecular tests to be performed by colleagues at the Cambridge University Haemato-oncology Diagnostic Service. These are very important to accurately sub-classify the disease. Treatment will very much depend on the specific type and extent of the myeloproliferative disorder that we diagnose.
We now understand much more about the molecular mechanisms that cause many of the myeloproliferative conditions and in some cases this has revolutionised the management of patients with these conditions. A good example is chronic myeloid leukaemia. This rare condition used to be inevitably fatal without stem cell transplantation, but now the vast majority of patients can be managed with out-patient therapy, taking tablet medication that specifically switches off the abnormal molecular switch that drives the disease.