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:
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:
- Age
- 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.
