A case of POEMS syndrome associated with reactive amyloidosis and Waldenström's macroglobulinaemia

Abstract

A 72-year-old Japanese man presented with a 5-year history of fatigue, night sweats, oedema of dorsum of foot, muscle weakness, and paresthesia of the lower extremities. On examination, the patient fulfilled the diagnostic criteria for POEMS syndrome, but was unusual in that he also had underling Waldenström's macroglobulinaemia with IgM κ. The M protein of POEMS syndrome is usually IgG and IgA with λ light chains. To our knowledge, there are only three cases of POEMS syndrome with IgM κ including our case. Autopsy findings revealed that small lymphocytes were more prominent than plasma cells in the bone marrow. These plasma cells were stained with IgM κ. Amyloid deposits identified by Congo red stain were detected predominantly in the vascular walls of the gastrointestinal tract, pancreas and kidney. This amyloid protein was classified as amyloid associated protein. This is the first case of POEMS syndrome associated with reactive amyloidosis and Waldenström's macroglobulinaemiae.