What is Waldenström’s Syndrome: Causes, Symptoms, Treatment

Waldenström’s Syndrome is the name given to a malignant disorder of the bone marrow and lymphatic tissues. This condition is a type of a lymphoma in which there is excessive numbers of a white blood cell called B lymphocytes.

With time and as the disease advances there is gradual accumulation of these white blood cells resulting in an increase of the antibody protein IgM. This increased production of IgM results in the blood getting abnormally thick. As a result, the blood is not able to move freely within the small blood vessels causing a variety of problems due to lack of adequate blood circulation through the body.

In some cases, the blood vessel may also rupture as a result of the thick blood that flows through it due to increased IgM. This tearing of the blood vessels may lead to bleeding from the nose, gums, and eyes as a result of Waldenström’s Syndrome.

The root cause of Waldenström’s Syndrome is still a matter of investigation and the exact cause has still not been found. In some cases, Waldenström’s Syndrome has been known to occur in families but such cases are extremely rare and there has been no proven link between the genetic makeup of an individual and Waldenström’s Syndrome.

The classic presenting feature of Waldenström’s Syndrome is the presence of symptoms of anemia with fatigue and lethargy. This is due to the loss of blood as a result of rupture of the blood vessels due to the blood being excessively thick.

Bleeding from the nose, gums, and the eyes is also common in Waldenström’s Syndrome. Peripheral neuropathy is yet another symptom of Waldenström’s Syndrome. Additionally, hepatosplenomegaly is also present in individual with Waldenström’s Syndrome. The symptoms are extremely variable in individuals with Waldenström’s Syndrome and depend on the thickness of the blood which is caused due to increased levels of IgM.

The diagnosis of Waldenström’s Syndrome is suspected when an individual presents with complaints of bleeding from the nose, gums, and eyes. A physical examination conducted will reveal an enlarged spleen and liver which will further point to Waldenström’s Syndrome.

This will result in a blood test being conducted which will reveal low red blood cell count and an elevated white blood cell count. Serum testing to check the IgM levels will show that it is significantly elevated thus confirming the diagnosis of Waldenström’s Syndrome.

As long as the individual is asymptomatic with a known diagnosis of Waldenström’s Syndrome, a wait and watch approach is adopted. Plasmapheresis is normally done to reduce the thickness of the blood so that it can flow more smoothly through the blood vessels.

In this process, the excess IgM is filtered out thus making the blood less thick. In cases where the disease condition advances then blood transfusion or platelet transfusion may need to be done.

Additionally, there are many chemotherapeutic agents that can be used for treatment of Waldenström’s Syndrome of which Cytoxan is most widely used for this purpose.

Additionally, rituximab has also shown good response towards treatment of Waldenström’s Syndrome. As of now, the latest medication to be approved by FDA is ibrutinib for treatment of Waldenström’s Syndrome.

References: