Waldenström's Macroglobulinemia
This disease is due to overproduction of cells that are like Plasma cells from the bone marrow. Under normal circumstances, these cells are in charge of producing immune globulins, the immune proteins that our body needs to fight infections. In case of Waldenström's Disease, the abnormal plasma cells produce abnormal and dysfunctional immune globulins. The kind of immune globulin that is produced in this disease can cause thickening of the blood and lead to a Hyperviscosity syndrome. This disease is basically diagnosed by detecting these abnormal immune globulins in the blood, or alternatively, by observing the abnormal cells in the bone marrow. This is a very rare and low grade cancer and is more commonly seen after age of 65.
Causes
The cause of this illness is unknown. The disease is named after Professor Jan Waldenström, a Swedish physician who described this illness.
Signs and Symptoms
Patients may not show any signs for quite sometime. This illness can present itself in many different fashions. Most patients, however, present with Hyperviscosity syndrome. As the disease progresses, it can cause any of the following problems:
- Anemia
- Low platelet count
- Frequent infections
- Fever
- Weight loss
- Enlargement of lymph glands
- Enlargement of Liver and spleen.
Diagnosis
The diagnosis is established by certain blood tests and also taking a biopsy of the bone marrow.
Treatment
Waldenström's Disease involves almost all of the bone marrow space in the body. As a result, the disease can only be treated with chemotherapy. Any patient who is under the age of 60, and otherwise in good health, should be considered for a bone marrow transplantation. For individuals who are older or can not tolerate the transplant, chemotherapy is most appropriate. If the disease causes a local problem in bones, like bone pain, or fractures, etc., radiation can be used to alleviate that problem.
The most commonly used chemotherapy regimens for treatment of Waldenström's Disease is Cladribine (Leustatin). This drug can induce a very good remission in great majority of patients.
Other drugs that might be used are : Melphalan , Prednisone, Cytoxan, Interferon
Remission
Refers to the response of any cancer to the treatment.
Complete remission refers to the situation where the disease disappears completely with the treatment
Partial remission refers to the situation where the disease shrinks but does not disappears completely with the treatment
The remission status is determined by measuring the immune globulin levels in the blood, as well as repeating the bone marrow tests.
Prognosis
Waldenström's Disease may remain silent for years. It may, however, gradually transition into more aggressive stage, or even transform into a Lymphoma. Prognosis of Waldenström's Disease depends on the response to chemotherapy.
Follow up
After completion of treatment, and in any combination that might have taken place, patients need to remain under surveillance for possibility of recurrence of cancer. Follow up shall be scheduled on a regular basis and initially every 1-3 months for 1-2 years. The frequency of follow up will depend on the condition of the patient and their disease. In each follow up visits, patients are examined and normally a blood test and evaluation of immune globulins should be done.
Treatment of recurrent disease will depend on the stage and extent of the reoccurred disease. Most patients are then treated with chemotherapy with same agents as mentioned above. Radiation therapy may be helpful in managing painful or symptomatic areas, where the cancer has spread to.
