indiacancer.org - Myelodysplastic Syndromes

	Myelodysplastic Syndromes

		What is the Bone Marrow?
		What are Myelodysplastic Syndromes?
		How do Myelodysplastic Syndromes present?
		How are Myelodysplastic Syndromes detected?
		What are staging and grading?
		How are Myelodysplastic Syndromes treated?
		What are the side effects of treatment for Myelodysplastic Syndromes?
		What is the importance of follow up?

	What is the Bone Marrow?

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.

What are Myelodysplastic Syndromes?

These are a rare group of diseases also known as a preleukaemia or smouldering leukaemia, and they are basically diseases of the bone marrow. In these diseases, the bone marrow fails to function normally and an inadequate number of blood cells which are normal, are produced.

Usually a disease of the older people, myelodysplastic syndromes may also be seen in younger people.

How do Myelodysplastic Syndromes present?

The usual presentation is in the form of a lack of mature red blood cells, which are oxygen carriers. This results in anemia. There are also very few infection-fighting white blood cells, resulting in recurrent infections which are hard to control. The number of platelets in the blood also may be subnormal. Platelets are cells in the blood which control clotting and if these cells are lacking in number, then the patient may bleed easily and spontaneously.

If any of these symptoms are present then the doctor should be seen as quickly as possible.

How are Myelodysplastic Syndromes detected?

Following a detailed history in which the following symptoms are detected, the doctor performs an examination and then does 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.

Depending on the dysplastic picture which is seen on the slide from the bone-marrow study, the doctor plans further treatment.

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 the 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 very 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, Bone scan or chest X-ray. There is really no clear-cut staging for myelodysplastic syndromes, but for treatment purpose they are often grouped into the following categories: Denovomyelodysplastic syndromes, which develop without any known cause. The patient has not received any kind of systemic chemotherapy or radiation for any other disease. Secondary myelodysplastic syndromes. These are those which develop following treatment by radiation or chemotherapy for some other disease. Previously treated myelodysplastic syndromes, which means that although the disease has been treated it has steadily got worse. Five types of myelodysplastic syndromes are known:
		Chronic myelomonocytic leukaemia.
		Refractory anaemia.
		Refractory anaemia with the ring sideroblasts.
		Refractory anaemia with excess blasts.
		Refractory anaemia with excess blasts and transformation.

How are Myelodysplastic Syndromes 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 hematologist, a medical oncologist and a radiotherapist. The plan of treatment is tailored to fit a patient's requirements. Treatment strategies. The bastion of treatment for myelodysplastic syndrome is blood transfusion or transfusion of formed elements.Formed elements are those parts of the blood which are the non-liquid portion. These are the cells which are there in the blood. Chemotherapy and biological therapy are also being tested out. The best attempt to control these 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). 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 denovomyelodysplastic syndromes, one of the following treatments may be entertained:
		Blood transfusion or treatment to release symptoms of the disease such as anaemia or bleeding.
		Chemotherapy or biological therapy.
		Allogenic BMT.
	Secondary myelodysplastic syndromes.
		Treatment by transfusions or other methods to relieve the symptoms of the disease.
		Chemotherapy or biological therapy may also be tried out.
	Previously treated myelodysplastic syndromes
		Symptomatic treatment only.
		They also may opt for chemotherapy or biological therapy.

What are the side effects of treatment for Myelodysplastic Syndromes?

	Unfortunately, treatment for cancer does cause damage to healthy tissues. Please ask the clinician about any possible side effects before any form of treatment commence. The various drugs used 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:
		Infections.
		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 (chemotherapist) 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 follow-up 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.