Pathophysiology and Treatments of Complications of Waldenström's Macroglobulinemia.

Abstract

Waldenstrom's macroglobulinemia (WM) or lymphoplasmacytic lymphoma is a B-cell malignancy characterized by lymphoplasmacytic cells in the bone marrow that secrete high amounts of immunoglobulin (Ig) M. The large pentameric structure of IgM leads to a variety of unique complications in WM, such as hyperviscosity syndrome, cryoglobulinemia and sensory neuropathy. Furthermore, malignant cells can infiltrate the central nervous system and lead to a variety of neurological complications, also known as Bing Neel Syndrome. Because of the unique pathophysiology of WM and these complications, their diagnostic work up and treatment regimens vary greatly. Given the rarity of the disease and their complications, there are little to no randomized controlled trials regarding treatments of these complications and, therefore, suggested treatment regimens are usually based on observational studies. In this case series, we will present three cases of WM, each with their own unique complication, and discuss the pathophysiology along with current and future treatment options for each of the complications presented.