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Hairy cell leukaemia (HCL) is a rare blood cancer, affecting around 0.3 people per 100,000 every year in the UK. A diagnosis of any cancer can be difficult but a diagnosis of a rare cancer can be even harder. You might find it challenging to relate to people and talk to people about your diagnosis.
What is it?
Hairy cell leukaemia (HCL) is a cancer of the blood and bone marrow. This rare type of leukaemia is indolent which means that it gets worse slowly or does not get worse at all. The disease is called hairy cell leukaemia because the leukaemic cells look "hairy" when viewed under a microscope in the lab.
Who does it affect?
HCL affects more men than women, and it occurs most commonly in middle-aged or older adults. Doctors aren’t sure what causes HCL, and there is no cure. HCL is considered a chronic disease because it may never completely disappear, although treatment can lead to a remission for years.
What are the main symptoms?
Some people have no signs or symptoms of HCL, but a blood test for another disease or condition may inadvertently reveal HCL. However, for most of the patients the most common symptom at first presentation is infection, which can occasionally be severe due to the underlying neutropenia (increased susceptibility to infection due to low neutrophil count). Other times people with HCL experience signs and symptoms common to a number of diseases and conditions but related to the bone marrow failure,
How is it diagnosed?
There are a number of tests used to diagnose HCL:
1) Physical exam.
Examination of the liver and the spleen . An enlarged spleen may cause a sensation of fullness in your abdomen that makes it uncomfortable to eat. Your doctor may also check for enlarged lymph nodes that may contain leukaemia cells although this is less likely in the classical HCL.
2) Blood tests.
Your doctor uses blood tests, such as the full blood count, to monitor the levels of blood cells in your blood. People with hairy cell leukaemia have low levels of all three types of blood cells — red blood cells, white blood cells and platelets. Another blood test called a peripheral blood film looks for HCL cells in a sample of your blood.
3) Bone marrow aspirate and trephine.
During a bone marrow biopsy you may have either a bone marrow aspiration or a bone marrow trephine biopsy. During a bone marrow aspiration, the doctor or nurse takes some bone marrow cells up into a syringe. A bone marrow trephine is when they remove a 1-2cm core of bone marrow in one piece. This sample is used to look for hairy cell leukaemia cells under the microscope.
4) Computerised tomography (CT) scan.
A CT scan shows detailed images of the inside of your body. Your doctor may order a CT scan to detect enlargement of your spleen and your lymph nodes.
Treatment may be advised for HCL patients with low blood cell counts, recurrent infections, or an enlarged spleen or lymph nodes. Treatment is most often with one of the purine analogue drugs - either cladribine (2-CdA) or pentostatin. In the UK, cladribine is the first choice. Up to 80%-90% of patients respond to these drugs, and the responses last more than five years in most patients.
If the leukaemia comes back, it is usually treated with a purine analogue again. Often the same drug will be used as was given the first time, especially if the leukaemia stayed in remission for a long time. Sometimes the monoclonal antibody, rituximab (Rituxan), will be given as well.
In rare cases, HCL may not respond to chemotherapy. Rituximab or interferon alpha, a type of biological therapy, may be helpful. If the patient is uncomfortable because of an enlarged spleen, removing the spleen by surgery (splenectomy) can often help relieve pain or other symptoms. Patients often need blood product support during chemotherapy treatment and they should receive irradiated blood products while on treatment with cladribine or pentostatin.
The prognosis is usually favourable with an overall survival of many years when treated with the drugs mentioned here. However the disease is not curable with conventional chemotherapy treatment.