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Macroglobulinemia

Macroglobulinemia is one type of cancer belongs to the category of non-Hodgkin’s lymphoma. It is indeed a slow cancer that attacks the immune system of the body.

Abnormal white blood corpuscles (lymphocytes) make a protein called monoclonal immunoglobulin (IgM) which is responsible for thickening of plasma. This in turn causes macroglobulinemia. When this protein is secreted in excess quantity, it affects the blood thickness, making it very thick.

Causes :

This disorder is caused mainly due to the thickening of blood plasma due to the condition of lymphophlasmacytic lymphoma. There will be excess production of IgM protein in the blood which is due to the lymphoma cells.

The blood becomes so thick (this condition is called hypervisocisity) that it cannot normally flow through tiny blood vessels.

This problem largely occurs in elderly persons but there are young people who suffer from this condition.

Symptoms :

Some of the major symptoms of Macroglobulinemia are bleeding of gums, dizziness, nausea, blurred vision, fatigue, Headache, change in mental status, bleeding in nose, numbness associated with tingling of fingers, toes and hands, skin rash, Weight Loss and partial vision loss.

Apart from the above, there would be change in skin color and fingers color for some people. There would be intense pain in the joints and swollen glands.

Diagnoses :

The doctor may first physically examine the body of the patient. He would then ask for laboratory tests for measuring blood profile which indicates the presence of IgM protein. He may ask the person to undergo biopsy on bone marrow for detecting the changes in lymphocytes.

Treatment :

It does not mean that all the persons who have IgM protein require immediate treatment. There are so many people who live without any symptoms and without taking any treatment for this problem. Treatment method depends on the intensity of symptoms and severity of the disorder.

Chemotherapy is done for destroying the abnormal cells so that bone marrow will not get disturbed anymore. It is also preferred by many patients to reduce the level of IgM protein in blood. Chemotherapy is given either intravenously or in the form of oral pills.

Plasma exchange is another method of treating this disorder. In this mode, unwanted IgM protein is washed out of the bloodstream so that the blood protein level is normalized, thus reducing the thickness. It requires 2-3 sessions for completing the course of treatment.

Biotherapy method is combined with chemotherapy to boost the immunity level to fight cancerous cells. Bone marrow transplantation needs to be done for some cases, depending on the condition of the disease.

Through the process of plasmapheresis unwanted elements of protein can be removed from the blood. This method can quickly control the IgM protein level in blood.

Drugs that are given for macroglobulinemia are Alkeran, Leukeran, rituximab, fludarabine or cytoxan. Many times, drugs are given in combination with chemotherapy.

It is necessary to follow up with your doctor in the right time till the course of treatment is completed. He would perform coagulation tests for checking the viscosity of blood and to monitor the levels of IgM protein in blood plasma.

Macroglobulinemia – Symptoms, Prognosis, Diagnosis, Treatment

What is Macroglobulinemia?

This is a very rare condition which involves the enlargement of the Spleen and liver linked to anemia – low blood count – bleeding nose as well as an atypical blood protein. This condition is also called Waldenstrom macroglobulinemia after the Professor who was the first to define individuals who were suffering with this condition. His portrayal contained many of the clinical structures of this disorder.

Macroglobulinemia is a kind of non-Hodgkin lymphoma.

Macroglobulinemia denotes growth within the cavity of the bone marrow of an abnormal population of lymphocytes, a kind of white blood cell. These lymphocytes in the bone marrow impede with the normal bone marrow role. If this growth of abnormal lymphocytes is not stopped, it can result in increasing symptoms which are related to low blood cell counts as well as the enlargement of the lymph glands. These enlarged lymph glands are most normally seen under the arms, in the groin or in the neck.

Macroglobulinemia Symptoms

There are numerous individuals who have this condition and have no symptoms with the condition being discovered purely by chance during routine blood tests. Others develop symptoms which are caused from the intrusion with blood flow to the fingers, skin, nose, toes as well as the brain that happen when a large quantity of macroglobulins thickens the blood. This thickening of blood is known as hyperviscosity syndrome. These symptoms include:

Bleeding from the skin and mucous membranes – such as the lining of the nose, mouth and digestive tract

Fatigue

Headache

Weakness

Dizziness

Confusion

Coma

The thickening of the blood can also intensify conditions of the heart and also increased pressure in the brain. The tiny vessels located in the back of eyes can become filled with blood as well as can bleed, resulting in retina damage and impaired eyesight.

Individuals with macroglobulinemia can have Swollen Lymph Nodes and liver and spleen enlargement because of the infiltration by the cancerous plasma cells. There are recurring bacterial infections which result from insufficient creation of normal antibodies and these can cause chills and fever. The weakness and fatigue with this disease is usually caused by the anemia which follows when cancerous plasma cells stops the normal blood-forming cells in the bone marrow from being produced. The infiltration of bones by the cancerous cells of plasma will also cause the loss of bone density – Osteoporosis – which weakens the bones and raises the risk of fractures.

There are some individuals who develop another condition which is known as cryoglobulinemia. This condition involves the creation of antibodies which clog up the vessels in temperatures which are cold.

Macroglobulinemia Diagnosis

Whenever macroglobulinemia is suspected blood tests are done. The three tests which are most useful are:

Serum protein electrophoresis

Measurement of immunoglobulins

Immunoelectrophoresis

Medical professionals also do other tests in the lab as well. For instance, they can check blood samples to determine if the number of white and red blood cells and platelets are normal. Additionally, a test named serum viscosity can be done to check the thickness of the blood. Clotting of the blood test results can be abnormal and there are other tests to detect cryoglobulins. Urine sample exams can show Bence Jones proteins which are pieces of atypical antibodies. A biopsy of the bone marrow can reveal if there are an increased number of plasma and lymphocytes cells, which is an aid in confirming the diagnosis of macroglobulinemia and the appearance of these cells helps to separate this illness from Multiple Myeloma.
X-rays will confirm any loss of bone density or osteoporosis. CT or computed tomography scans can reveal any Enlarged Liver, spleen or lymph nodes.

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Macroglobulinemia Treatment

Macroglobulinemia requires chemotherapy with drugs which attack the abnormal cells to reduce their effect on normal and healthy bone marrow and also reduce the level of abnormal protein produced. Drugs which are used alone or also in combination include:

Chlorambucil

Fludarabine

Melphalan

Cyclophosphamide

Rituximab

Thalidomide

Lenalidomide

Bortezomib

These drugs can be given intravenously or orally. None of these drugs can cure this condition only slow down the progression. There is research on different combinations of medications as is research into the possibility of non-chemotherapy treatments.

While on chemotherapy, medical professionals will closely monitor levels of abnormal protein in the urine and blood. Periodically samples of bone marrow will be obtained to assess the number of atypical lymphocytes. Using this information physicians can observe progress and adjust treatment as needed.

Any drop in the level of atypical protein normally means that the treatment is effective. A protein level which is stable indicates that the disease is stable but not gone. Rarely will the protein disappear totally from the urine or blood.

If the thickening of the blood is causing complications or problems, a plasma exchange can be used to wash the plasma out of the blood system – cleansing the plasma. This is an effective method normally required only for a short time to lower levels of blood protein.

Macroglobulinemia is a chronic disease that cannot be cured but can be managed long-term with treatment. Many individuals have good results and can return to activity levels which are normal.

This is a very stressful disease both for the patients as well as the family because of the worry about the effects of the disease – this is normal. Counseling is advised to help with the worries and fears and the emotional distress of this disorder.

Macroglobulinemia Prognosis

The prognosis for this type of cancer usually depends on the following:

General health

Genetic or hereditary makeup

Males especially those over the age of 60 as well as those with severe anemia have the lowest rates of survival. The Revised European American Lymphome or REAL system of classification is used to measure prognosis and it gives macroglobulinemia a fairly good prognosis after treatment, with the average being a survival rate of 50 to 70 percent after 5-years. But many individuals live much longer. Approximately 16 to 23 percent of individuals die of causes which are unrelated.