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Providing support to anyone affected by blood cancer
When someone says ‘blood cancer’, many people will think ‘leukaemia’. However, there are a range of types and sub-types of blood cancer, including lymphoma and myeloma. In this article, we’ll be taking a look at five rare blood cancers to try and raise awareness.
Hairy cell leukaemia (HCL) affects around 0.3 people per 100,000 every year in the UK. That’s just 0.003%. It’s a rare type of leukaemia and is indolent, meaning that it can get worse slowly or never progress at all.
It’s caused by an excess number of abnormal B lymphocytes (white blood cells that produce antibodies) which means the patient cannot fight infection properly. The bone marrow is then not able to make enough normal blood cells to compensate for the abundance of abnormal lymphocytes.
The name derives from the fact that the lymphocytes have fine projections on the surface that look like hairs under the microscope. So, they look like ‘hairy’ cells.
This type of leukaemia is more prevalent in the older generation and men. It’s also chronic, meaning that it can’t be cured. However, it does respond especially well to chemotherapy and so can be kept under control. This means that many hairy cell sufferers can expect a normal or near-normal life span.
Large granular lymphocytic leukaemia (LGLL) is very rare and occurs in two forms: T-cell (T-LGLL) and natural killer (NK-LGLL). LGLL gets its name from the fact that the affected enlarged lymphocytes (T-cell or NK) contain granules that can be seen under a microscope.
There are three factors that can contribute to your likelihood of developing LGLL:
Around half of LGLL patients are asymptomatic and so do not need immediate treatment; this is called ‘watch and wait’. However, if a patient is displaying the common symptoms of repeated infections and anaemia, then treatment will begin immediately.
Unlike other cancers, LGLL is not actually treated with cell-killing chemotherapy drugs. Instead, patients are treated with the following:
Whilst LGLL is not considered curable, it often responds well to treatment, meaning that patients have a normal or near-normal life expectancy and quality of life.
Acute promyelocytic leukaemia (APL or APML) is a rare sub-type of acute myeloid leukaemia (AML) that affects the promyelocytic cells, which assist in the production of blood. However, APL is treated in a very different way to AML, as AML treatment can cause serious risks to an APL patient’s clotting system.
APL makes up 10-12% of AML cases, and the median age of onset is about 40. The cause of most cases of APL are unknown. It is sometimes seen in people who have been treated for other forms of cancer; this only affects a very small percentage of cancer patients. This is known as therapy-related APL (t-APL) and accounts for about 1 in 10 cases of APL.
However, often APL is characterised by a merging of two genes – PML and RARA – which are usually found on two different chromosomes. In APL, the two chromosomes swap over part of their DNA, which joins the PML and RARA genes together. This faulty gene affects the promyelocytic cells’ ability to develop, thus causing them to become malignant.
Overall, APL responds well to treatment and many patients have a good chance of being cured. The standard line of treatment is chemotherapy and a drug called ATRA, which blocks the abnormal PML-RARA ‘fusion gene’, allowing the promyelocytic cells to mature properly.
We also have a booklet available on acute promyelocytic leukaemia here.
Burkitt lymphoma (BL) is a variant of B-cell non-Hodgkin lymphoma that affects 1 in every 30-50 people diagnosed with a B-cell non-Hodgkin lymphoma.
BL is recognised as the fastest growing human tumour and is associated with impaired immunity and can be fatal if left untreated.
There are three types of BL:
Outside of Africa, both children and adults can be affected by the sporadic type. It makes up over a third of all the lymphomas seen in children, and is more prevalent in males – more than two-thirds of adults with BL are men.
BL usually responds well to treatment, and many patients have a good chance of being cured.
Myeloma, also commonly known as multiple myeloma, is a cancer of the plasma cells in the bone marrow. Plasma cells are types of white cells responsible for producing antibodies (immunoglobulin).
When plasma cells become old, new ones are created to take their place in an orderly and controlled method. However, in myeloma, this process becomes out of control and large amounts of abnormal, malignant plasma cells are produced, releasing only one type of abnormal antibody, known as paraprotein - it has no useful function and can’t fight infection.
The abnormal plasma cells continue to multiply and overwhelm the system, causing a lack of normal red and white blood cells. The paraprotein produced acts as a marker for doctors to be able to spot the presence of myeloma. However, in about 3 out of every 100 people with myeloma (3%), the malignant plasma cells secrete little or no paraprotein.
This can sometimes delay a diagnosis of myeloma, but otherwise patients with non-secretory myeloma still undergo the same treatment and have no difference in survival rates.
There is no specific cause of myeloma, but there are a number of contributing factors:
Unlike many other cancers, including some blood cancers, myeloma does not cause the growth of tumours. It’s also chronic and prone to relapsing and remitting, meaning it can’t be cured but can have stages of relative inactivity where treatment isn’t required.
How you can help
The vague signs and symptoms of blood cancer in general, and the number of rare types, mean that it’s imperative people know the signs of blood cancer.
If you’d like to help or to inform yourself, then download one of our symptoms cards here.