indiacancer.org - Hairy Cell Leukaemia

Hairy Cell Leukaemia

		What are Lymphocytes? What is the Bone Marrow? What is the Lymphatic System?
		What is Hairy Cell Leukaemia?
		How does Hairy Cell Leukaemia present?
		How are Hairy Cell Leukaemias detected?
		What are staging and grading?
		How is Hairy Cell Leukaemia treated?
		What are the side effects of treatment for Hairy Cell Leukaemias?
		What is the importance of follow up?

What are Lymphocytes? What is the Bone Marrow? What is the Lymphatic System?

The lymphocytes are a particular type of infection-fighting white blood cells which are formed in the bone marrow and which circulate along with the blood.

The bone marrow is a spongy tissue, which is found mainly in the large bones in the body. The bone marrow is responsible for production of most of the blood's formed elements, which include the red blood cells (carriers of oxygen to all tissues of the body), white blood cells (fighters of infection), and platelets (clotters of blood).

Usually, the bone marrow makes cells called blasts that ultimately mature into various types of blood cells, and each of these types of blood cells have specific jobs to do in the body.

Lymphocytes are cells circulating in the lymph which is a colorless watery fluid present in lymph vessels. Lymph vessels are transparent thin tubes, which traverse all the systems in the body akin to the circulatory system of the blood. At various points along their route, they come to junctions or stops, which are known as lymph nodes. Lymph nodes are small, bean-shaped organs, which harbor clusters of lymphocytes. They also help to a smaller extent in the production of lymphocytes. Lymph nodes are clustered in large groups in the neck, in the back of the abdomen, in the pelvis and under the armpits. The lymph nodes and the spleen, the thymus, the tonsils, and the bone marrow are all part of the reticuloendothelial system.

The spleen is an organ located in the left side of the upper abdomen that makes lymphocytes and filters senescent or old red blood cells from the blood.

The thymus is a small organ, which is located beneath the breastbone and is most prominent during childhood.

The tonsils are groups of lymphoid tissue, which are present in the back of the throat and along the sides of the throat.

What is Hairy Cell Leukaemia?

Hairy cell leukaemia is a cancer found in the blood and the bone marrow. It is called by this name because it is a leukaemia in which the cancer cells look hairy when examined under the microscope.

How does Hairy Cell Leukaemia present?

Beside the standard symptoms of leukaemia, the classical hallmarks of hairy cell leukaemia include the collection of leukaemia cells in the spleen, so that the spleen swells up in size.
There may be very few normal white blood cells in the blood because the leukaemia cells have totally overtaken and invaded the bone marrow. This results in a severe drop in the immunity or the ability of the individual to resist infection, which results in infection which does not go away.
The person may also have severe tiredness.

How are Hairy Cell Leukaemias detected?

If any of these symptoms or more symptoms which can be described by a doctor present, then a visit to him is mandatory and this would include a physical examination and certain blood tests.

Blood tests may be quite thorough and detailed and quite a few of them may be done because leukaemias have to be absolutely properly classified.

The other test, which a doctor may have to perform is a bone marrow biopsy, in which a needle is inserted into a bone, usually the breastbone or one of the hipbones and a small amount of bone marrow is taken out and looked at under the microscope.

The doctor also may have to order a lumbar puncture, in which a needle is inserted through the back to take a small sample of the cerebrospinal fluid, which is the lubricating and nourishing fluid surrounding the brain and the spinal cord. Once the cells and the type of leukaemia have been adequately classified, then the treatment strategies can be mapped out.

Additional tests may also be ordered by the doctor, such as imaging investigations including an ultrasound of the abdomen or a CT scan of the abdomen, a CT scan of the chest or the brain or an MRI and chest x-rays.

What are staging and grading?

These are terms with which you must become familiar, if you are dealing with any form of cancer.

When we stage a cancer, we try to establish carefully degree of spread of the cancer and if indeed there is spread, to what extent and involving which organs.

The grading of a cancer is a microscopic issue, in which the pathologist lets you know how aggressive this particular type of cancer is; well differentiated means less aggressive, moderately differentiated intermediately aggressive and poorly differentiated more aggressive.

Both grading and staging help your clinician to establish the prognosis or how serious your disease is and what is going to be the likely outcome.

Staging may require additional imaging tests such as CT Scan, MRI, Sonography of the abdomen, IVU, Bone scan or chest X-ray.

There is no staging for hairy cell leukaemia. Patients are basically grouped together depending whether they have been treated before or not.

Untreated hairy cell leukaemia is that which has never been treated for the leukaemic pathology. Treatment may have been given for the effects of leukaemia or for the infections but never to treat the leukaemia per se.

Progressive hairy cell leukaemia Post operatively, although the spleen has been removed, the leukaemia keeps getting worse.

Refractory hairy cell leukaemia is that which has been treated but no longer shows any response to therapy.

How is Hairy Cell Leukaemia treated?

	A lot depends on the patient's constitution, the grade of his disease and its staging. Patients are usually treated by a team of specialists which utilizes a multipronged approach. This includes an oncosurgeon and a medical oncologist. Treatment strategies. Some patients have such few symptoms after they develop hairy cell leukaemia, that initially the doctor may decide to opt for a wait-and-watch policy. The primary treatments which are used include chemotherapy, biological therapy and surgery. Bone marrow transplants are also an additional option. As far as surgery is concerned, this is primarily to remove the spleen as it is greatly enlarged and this surgery is called a splenectomy. The best attempt to control leukaemia cancer cells circulating in the body and lodged at various places is by using systemic therapy (therapy which affects the entire systems of the body) which is in the form of injections or oral drugs. This form of treatment is called chemotherapy. This form of treatment is given in a cyclical manner (each set of drugs is repeated usually after every 3-4 weeks.) Chemotherapy can also be used in combination with surgery, radiotherapy or both, either before or after. Biological therapy employs Biological Response Modifiers (BRM's), which are substances that use the body's own immune system, either directly or indirectly, to fight cancer or to lessen the side effects of the anti cancer drugs. Some examples of BRM's are interferon-alfa and interleukin-2. Bone marrow transplantation is an option in which the entire bone marrow of the patient is replaced with healthy bone marrow. First of all the bone marrow in the body is destroyed with high-dose chemotherapy in association or without radiation therapy. The healthy bone marrow is then taken from another person whose tissue has the same HLA matching as the patient's. HLA is a kind of tissue matching, which is required to be done before any kind of tissue is transferred from a donor to a recipient in order to prevent a reaction and the donor's body from rejecting the entire tissue sample. The donor may be an identical twin, which always produces the best match, or a sibling such as a brother or sister, or a totally unrelated person. The healthy marrow from the donor is given to a patient just like saline, that is, through a needle in the vein and the marrow replaces the marrow that has been destroyed. A bone marrow transplant or a BMT using marrow from a relative or person not related to the patient is called an allogenic BMT. Autologous BMT is a type of transplant in which the bone marrow is taken from the patient before radical chemotherapy, and then is treated with drugs to kill any cancer cells. Then the marrow is cryogenically frozen to save it, and high-dose chemotherapy is given with or without radiation to the patient to destroy the remaining marrow. The frozen marrow is now thawed out and reintroduced into the patient through a needle in a vein. Maximum chances for recovery occur if the doctor chooses a hospital that has done a significant number of BMTs with very less evidence of either septicemia, infection, or rejection. Yet another type of autologous transplant is called a peripheral blood stem cell rescue transplant. In this, the patient's blood is passed through a machine that removes the immature cells from which all blood cells develop (the stem cells) and then returns the blood to the patient. This procedure, known as leukapheresis, usually takes 3 to 4 hours to complete. The stem cells are now treated with drugs to kill any cancer ingredient in them and are then cryogenically frozen until they can be transplanted to the patient. This may be done in association or disassociated from an autologous bone marrow transplant. For untreated hairy cell leukaemias, one of the following options may be entertained:
		If there is no symptomatology, a wait-and-watch policy may be adopted by the doctor until the leukaemia signs start getting away.
		Biological therapy.
		Chemotherapy.
		Splenectomy.
	For progressive hairy cell leukaemia, one of the following options may be entertained:
		If there is no response to biological therapy, chemotherapy may be an option.
		Variant experimental therapy may be tried out.

What are the side effects of treatment for Hairy Cell Leukaemias?

	Unfortunately treatment for cancer does cause damage to the healthy tissues. Please ask the clinician about any possible side effects before any form of treatment commences. The various drugs in chemotherapy cause a varied spectrum of side effects. The severity and variability of these symptoms are very individualistic, changing from patient to patient. Some of the common side effects are:
		Skin rashes.
		Loss of hair.
		Diarrhea.
		Vomiting.
		Tingling and numbness in the fingers and toes.
		Hearing loss.
	Most are temporary and recede after therapy is over. Hair growth gradually starts after cessation of chemotherapy. The medical oncologist will usually be able to reduce the severity and spectrum of these side effects. The psychology of the patient is very important during therapy. Better psychologically prepared the patient is, better are his chances of having decreased side effects. The patient should drink large quantities of water while the therapy is in progress, and preferably avoid uncooked or raw food. Water should be filtered or boiled. Any relative or person who has any sort of infection such as common cold should be asked to desist from coming close to the patient. Biological therapy. The side effects commonly encountered with this therapy are as follows:
		Low grade fever.
		Rashes and bruises.
		Nausea and Vomiting (usually mild).
	All these problems disappear on cessation of therapy. The riskiest problem as far as bone marrow transplantation is concerned is GVHD or graft versus host disease. In this, the donated bone marrow attacks the patient's tissues and blood, and causes severe reactions ranging from effects on the liver, to the skin and to the digestive system. GVHD can range from being very mild to very severe and can occur any time after the transplant, even many years later. Drugs are usually given along with GVHD, and subsequently in the years following the transplant in order to prevent GVHD sort of reactions. The patient's also face an increased risk of bleeding, infection, or other side effects associated with the immunocompromised.

What is the importance of follow up?

The importance of followup cannot be overemphasized. It is a fatal fallacy to assume on the patient's part that once the primary or the first phase of treatment is over, then he or she is cured. It must be stressed here that cancer has a notorious proclivity of coming back again and again. The only way to fight this is to pick up any problem when it is still early, so that it can be nipped in the bud. And the only way to do this is to follow up when the doctor calls you for the next visit. Each such visit will include a detailed history, a physical examination (including an internal i.e. a rectal or vaginal examination), a chest x-ray, an ultrasonography or a CT scan, and various other blood studies as indicated.