About hairy cell leukaemia?
HCL is one of the lymphoproliferative disorders of which the most common is chronic lymphocytic leukaemia (CLL). HCL is classified as a chronic leukaemia meaning it develops slowly, often over many years.
Hairy cell leukaemia: the basics
How common is hairy cell leukaemia?
HCL is a rare form of leukaemia and accounts for only 2% of all leukaemias diagnosed.
Who gets hairy cell leukaemia?
HCL is more common in men than women and usually occurs in middle age (the median age of onset is 50). It does not occur in children and is very rare in adolescents.
Causes hairy cell leukaemia
The causes of HCL remain unknown but it is thought to result from damage to one or more of the genes that normally controls blood cell development. We do know that it is not contagious or inherited or passed down through our genes.
Symptoms of hairy cell leukaemia
Because HCL develops slowly many people don’t have any symptoms, particularly in the early stages. The disease is usually picked up during a routine blood test.
Other people may go to see their GP because they have some troubling symptoms of their disease. Symptoms arise from the increasing number of abnormal blood cells in the bone marrow and blood, and the decreasing number of normal blood cells. Possible symptoms may include:
- anaemia, due to a lack of red cells causing persistent tiredness, dizziness, paleness, or shortness of breath when physically active
- frequent or repeated infections and slow healing, due to a lack of normal white blood cells
- pain or discomfort under the ribs on the left side, due to an enlarged spleen
- painless swelling of the lymph nodes (glands) in your neck, under your arms or in your groin. This is usually a result of lymphocytes accumulating in these tissues.
Some of the symptoms described above may also be seen in other illnesses, including viral infections. So, most people with these symptoms don’t have leukaemia. However, it is important to see your doctor if you have any unusual symptoms, or symptoms that persist much longer than expected so that you can be examined investigated properly.
Diagnosis of hairy cell leukaemia
When you see your doctor about any of your symptoms, they will probably take a full medical history, asking questions about your general health, any illness or surgery you have had previously. They may also give you a full physical examination and order further tests. HCL is diagnosed by using all of this information and examining samples of your blood and bone marrow.
Full blood count
The first step in the diagnosis is a simple blood test called a full blood count (FBC) or complete blood count (CBC). This involves taking a sample of blood from a vein in your arm being sent to the laboratory for investigation. Most people with HCL have a low red cell and platelet count. Many of the white blood cells may be abnormal leukaemic blast cells and the presence of these blast cells suggests you have HCL.
A diagnosis needs to be confirmed by examining your cells in your bone marrow.
Bone marrow examination
If the results of your blood tests suggest that you might have HCL, a bone marrow biopsy may be required to help confirm the diagnosis. A bone marrow biopsy involves taking a sample of bone marrow (usually from the back of the hip bone) and sending it to the laboratory for examination under the microscope. The bone marrow biopsy may be done in the haematologist’s rooms, clinic or day procedure centre and is usually performed under local anaesthesia with sedation given either by tablet or through a small drip in your arm.
The sample of bone marrow is examined in the laboratory to determine the number and type of cells present and the amount of haematopoiesis (blood forming) activity taking place there.
Further testing
Once a diagnosis of HCL is made, blood and bone marrow cells are examined further using special laboratory tests. These include cytogenetic, immunophenotyping and immunoglobulin (antibody) level tests. These tests provide more information about the exact type of disease you have, the likely course of your disease and the best way to treat it.
Other tests
These may be conducted to provide information on your general health and how your vital organs are functioning. These include a combination of further blood tests and imaging tests (x-rays, scans and ECG). These results will provide a baseline of your disease and general health, which will be compared with later results to assess how well you are progressing and responding to treatment.
Treatment of hairy cell leukaemia
In recent years effective drug treatments for HCL have been developed.
Chemotherapy
Chemotherapy is the main form of treatment for HCL. The aim of treatment is to destroy leukaemic cells and induce a remission. This means that there is no evidence of leukaemic cells in the blood and bone marrow and that normal blood cell production and normal blood counts are restored. A specific type of chemotherapy drug used is called a purine analogue. There are different variants of this type of chemotherapy available and your doctor will decide the best option for you.
Chemotherapy is usually given in several cycles (or courses) of treatment with a rest period of a few weeks in between each cycle. If a relapse of your disease does occur there is a very high probability that further treatment will be successful. Study and clinical trials that have been undertaken examining the use of targeted therapy most often in patients who have relapsed, have shown promising results. Your specialist will discuss treatment options with you if relapse occurs.
Interferon
Prior to the use of chemotherapy in successfully treating HCL, interferon was widely used. Interferon is now mainly used for patients who have low level blood counts as chemotherapy can lower these levels further.
Splenectomy
Patients who have their spleen removed (splenectomy) will find it relieves the discomfort caused by the enlargement and it may also defer the need for other treatments in the short term. After a splenectomy a patient is more susceptible to infections and will need to see their doctor immediately if they are unwell.
Side effects of hairy cell leukaemia treatment
All treatments can cause side effects. However, the type and severity will vary between individuals, depending on the type of treatment used and how an individual responds to it. In general, more intensive treatment is associated with more severe side effects. It is important to report any symptoms you are having to your doctor or nurse. In most cases they can be treated and are reversible.
When to contact your doctor or hospital
As a general rule, while you are having treatment you will need to contact your doctor or hospital immediately if you have any of the following:
- a temperature of 38oC or over (even if it returns to normal) and/or an episode of uncontrolled shivering (a rigor)
- bleeding or bruising, for example blood in your urine, faeces, sputum, bleeding gums or a persistent nose bleed
- nausea or vomiting that prevents you from eating or drinking or taking your normal medications
- severe diarrhoea, stomach cramps or constipation
- coughing or shortness of breath
- a new rash, reddening of the skin, itching
- a persistent headache
- a new pain or soreness anywhere
- if you cut or otherwise injure yourself
- if you notice pain, swelling, redness or pus anywhere on your body.
What are the side effects of hairy cell leukaemia treatment?
Chemotherapy kills cells that multiply quickly, such as leukaemic cells. It also causes damage to fast-growing normalcells, including hair cells and cells that make up the tissues in your mouth, gut and bone marrow. The side effects of chemotherapy occur as a result of this damage.
These include:
Effects on the bone marrow
Chemotherapy affects the bone marrow’s ability to produce adequate numbers of blood cells. As a result, your blood count (the number of white cells, platelets and red cells circulating in your blood) will generally fall within a week of treatment. The length of time it takes for your bone marrow and blood counts to recover mainly depends on the type of chemotherapy given.
When your platelet count is very low (thrombocytopenic) you can bruise and bleed more easily. During this time it is helpful to avoid sharp objects in your mouth such as chop bones or potato chips as these can cut your gums. Using a soft toothbrush also helps to protect your gums. In many cases a transfusion of platelets is given to reduce the risk of bleeding until the platelet count recovers.
If your red blood cell count and haemoglobin levels drop you will probably become anaemic. When you are anaemic you feel more tired and lethargic than usual. If your haemoglobin level is very low, your doctor may prescribe a blood transfusion.
Risk of Infection
The point at which your white blood cell count is at its lowest is called the nadir. This is usually expected 10 to 14 days after having your chemotherapy. During this time you will be at a higher risk of developing an infection. At this stage you will also be neutropenic, which means that your neutrophil count is low.
Neutrophils are important white blood cells that help us to fight infection. While your white blood cell count is low you should take sensible precautions to help prevent infection. These include avoiding crowds, avoiding close contact with people with contagious infections (for example colds, flu, chicken pox) and only eating food that has been properly prepared and cooked. If you do develop an infection you may experience a fever, which may or may not be accompanied by an episode of shivering where you shake uncontrollably. Infections while you are neutropenic can be quite serious and need to be treated with antibiotics as soon as possible. Sometimes your doctor may decide to use a drug to help the recovery of your neutrophil count. This drug works by stimulating the bone marrow to increase the production of neutrophils and is usually given as an injection under the skin (subcutaneous).
Nausea and vomiting
Nausea and vomiting are often associated with chemotherapy and some forms of radiotherapy. These days however, thanks to significant improvements in anti-sickness (anti-emetic) drugs, nausea and vomiting are generally very well-controlled. You will be given anti-sickness drugs before and for a few days after your chemotherapy treatment. Be sure to tell the nurses and doctors if the anti-emetics are not working for you and you still feel sick. Some people find that eating smaller meals more frequently during the day, rather than a few large meals, helps to reduce nausea and vomiting. Many find that eating cool or cold food is more palatable, for example jelly or custard. Drinking ginger ale or soda water and eating dry toast may also help if you are feeling sick.
Changes in taste and smell
Both chemotherapy and radiation therapy can cause changes to your sense of taste and smell. This is usually temporary but in some cases it lasts up to several months.
Mucositis
Mucositis, or inflammation of the lining of the mouth, throat or gut is a common and uncomfortable side effect of chemotherapy and some forms of radiotherapy. It usually starts about a week after the treatment has finished and goes away once your blood count recovers, usually a couple of weeks later. During this time your mouth and throat could get quite sore. Soluble pain medication and other topical drugs (ones that can be applied to the sore area) can help. If the pain becomes more severe, stronger pain killers might be needed. It is important to keep your mouth as clean as possible while you are having treatment to help prevent infection. Avoid commercial mouthwashes as these are often too strong or they may contain alcohol, which will hurt your mouth.
Bowel changes
Chemotherapy can cause some damage to the lining of your bowel wall. This can lead to cramping, wind, abdominal swelling and diarrhoea. Be sure to tell the nurses and doctors if you experience any of these symptoms. If you develop diarrhoea, a specimen will be required from you to ensure that the diarrhoea is not the result of an infection. After this you will be given some medication to help stop the diarrhoea and/or the discomfort you may be feeling. It is also important to tell the nurse or doctor if you are constipated or if you are feeling any discomfort or tenderness around your anus when you are trying to move your bowels. You may need a gentle laxative to help soften your bowel motion.
Hair loss
Hair loss is unfortunately a very common side effect of chemotherapy and some forms of radiotherapy. However, it is usually only temporary. The hair starts to fall out within a couple of weeks of treatment and tends to grow back three to six months later. Avoiding the use of heat or chemicals and only using a soft hairbrush and a mild baby shampoo can help reduce the itchiness and scalp tenderness that can occur while you are losing your hair. You need to avoid direct sunlight on your exposed head (wear a hat) because chemotherapy (and radiotherapy) makes your skin even more vulnerable to the damaging effects of the sun (like sunburn and skin cancers). Remember that without your hair your head can get quite cold so a beanie might be useful. Hair can also be lost from your eyebrows, eyelashes, arms and legs.
Fatigue
Most people experience some degree of tiredness in the days and weeks following chemotherapy and radiotherapy. Getting plenty of rest and a little light exercise each day may help to make you feel better during this time. It is important to listen to your body and rest when you are tired.
Fertility
Some types of chemotherapy and radiotherapy maycause a temporary or permanent reduction in your fertility. It is very important that you discuss any questions or concerns you might have regarding your future fertility with your doctor, if possible beforeyou commence treatment. In women, some types of chemotherapy and radiotherapy can cause varying degrees of damage to the normal functioning of the ovaries.
In men, sperm production can be impaired for a while but the production of new sperm may become normal again in the future. There are some options for preserving your fertility, if necessary, while you are having treatment for leukaemia.
Early menopause
Some cancer treatments can affect the normal functioning of the ovaries. This can sometimes lead to infertility and an earlier than expected onset of menopause, even at a young age. The onset of menopause in these circumstances can be sudden and, understandably, very distressing. Hormone changes can lead to many of the classic symptoms of menopause. Some women experience decreased sexual drive, anxiety and even depressive symptoms during this time. It is important that you discuss any changes to your periods with your doctor or nurse.
Body image, sexuality and sexual activity
Hair loss, skin changes and fatigue can all interfere with how you feel about yourself as a man or a woman and as a ‘sexual being’. During treatment you may experience a decrease in libido and it may take some time for things to return to ‘normal’. It is perfectly reasonable and safe to have sex while you are on treatment or shortly afterwards, but there are some precautions you need to take. It is usually recommended that you or your partner do not become pregnant as some of the treatments given might harm the developing baby. As such, you need to ensure that you or your partner uses a suitable form of contraception. Partners are sometimes afraid that sex might in some way harm the patient. This is not likely as long as the partner is free from any infections and the sex is relatively gentle. Finally, if you are experiencing vaginal dryness, a lubricant can be helpful.
Caring for someone with hairy cell leukaemia
We have a range of information and resources that may help when you are caring for someone with hairy cell leukaemia.
