Pearl
Like a “perfect storm”, Waldenstrom Macroglobulinemia (WM) is an unusual, rare hematologic malignancy that is a cross between lymphoma and multiple myeloma
This storm generates an overproduction of the largest immunoglobulin, IgM, which causes the patient's blood to thicken, called hyperviscosity
Essentially, this overproduction of IgM is causing a traffic problem in the blood, triggering the normal 6 lane highway to come to a crawl
Hyperviscosity Syndrome (HVS) is an oncologic emergency that presents with neurological symptoms, visual changes, and mucosal bleeding
Patients with HVS can often be dehydrated and anemic, but they should not be treated with a blood transfusion because it can increase serum viscosity and worsen symptoms
Fluids are the best initial management, along with plasmapheresis for short term definitive therapy to reverse symptoms. Long term definitive treatment of HVS involves treatment of the underlying WM.
💡 A pearl to keep in your back pocket
If the IgM is >/=4000, we should NOT give rituximab, a common treatment for WM (since B cells are involved in the pathophysiology). This drug can actually cause a tumor flare when levels are this high which can increase hyperviscosity symptoms. |