Treatment for Enlarged Spleen, Plasmapheresis, and Other Control Measures

 

Treatment Plasmapheresis, or plasma exchange, is used to thin the blood and provide rapid symptom relief from hyperviscosity syndrome.

 

 

Waldenstrom's macroglobulinemia is classified as an "orphan" disease. Orphan diseases are so rare that few, if any, research funds are available. Current treatment options for Waldenstrom's macroglobulinemia are based on research into similar disorders, such as chronic lymphocytic leukemia  and multiple myeloma.

 

The intent of WM treatment is not to cure the disease. In fact, at present no cure exists for Waldenstrom's macroglobulinemia. Instead, treatment aims to monitor the disease's progression and provide symptom relief.

Chemotherapy is usually the first treatment option for most WM patients. Purine nucleoside analogs such as fludarabine and cladribine are often prescribed. The two medications may be used alone or in combination. The majority of people treated with purine nucleoside analogs respond favorably to the medication.

 

A second variety of chemotherapy drugs called alkylating agents may also be used. Alkylating agents include chlorambucil, melphalan, and cyclophosphamide, and may be prescribed with corticosteroids.

 

Chemotherapy medications are systemic drugs: they affect the entire body. Chemotherapy works by targeting and killing fast growing cells, such as cancer cells. Healthy cells that grow quickly are also affected by chemotherapy, however, resulting in a number of possible side effects, such as hair loss.

 

Although nucleoside analogs and alkylating agents often generate positive responses, most people eventually build up a resistance to these medications, making long-term chemotherapy ineffective.

Hyperviscosity, or thick blood, is one of the most common complications of Waldenstrom's macroglobulinemia, and can result in a number of symptoms and complications. A process called plasmapheresis, or plasma exchange, is used to thin the blood and provide rapid symptom relief from hyperviscosity syndrome.

 

Plasmapheresis works by filtering the blood through a special machine that breaks the blood down into its components. The blood is delivered to the machine through a catheter inserted into a vein. During this process plasma containing IgM antibodies is removed, and replaced with a sterile saline solution. The blood is then returned to the body.

 

Plasmapheresis is not an answer to hyperviscosity, however: after treatment, the blood will once more begin to thicken. The treatment does provide symptom relief. If hyperviscosity is affecting vision, prompt plasmapheresis can prevent blindness.

People with WM may experience symptoms and discomfort due to an enlarged spleen. Splenic radiation therapy may be used to reduce symptoms. The radiation shrinks the enlarged spleen. Splenectomy (surgical removal of the spleen) may be necessary for symptom relief in more advanced cases.

In the later stages of Waldenstrom's macroglobulinemia, the high levels of irregular B cells and IgM antibodies cause imbalances in the blood. Anemia, low platelet counts and insufficient white blood cells (WBC) may occur. Blood transfusions may be necessary to restore red blood cell and platelet counts. Antibiotics can help treat and prevent infections.

Waldenstrom's macroglobulinemia is benefiting from cancer clinical trials. Stem cell transplantation shows promise as a treatment for several types of lymphoma (a related blood cancer). Stem cells are produced by the bone marrow, and mature into the different types of blood cells.

 

High dose chemotherapy is used to kill existing stem cells and temporarily suppress stem cell production in the bone marrow. This kills both healthy and abnormal cells. After this procedure, healthy stem cells are injected into the blood stream. These cells may have been "harvested" from the patient or someone else and are cryogenically frozen until needed. The new stem cells then mature into healthy blood cells.

 

Because transplantation of stem cells is still an experimental procedure for WM treatment, it is an approach used when traditional methods for controlling symptoms are ineffective.

As treatment options for WM are limited, people with the disease may wish to explore therapies under investigation by participating in clinical trials. Several promising therapies are under investigation:

 

 

 

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