Intestinal Lymphangiectasia as a Rare Myeloma-Defining Event

Multiple myeloma is a clonal plasma cell disorder with a pre-malignant phase in the form of monoclonal gammopathy of uncertain significance (MGUS) and smoldering myeloma. Progression from MGUS or smoldering myeloma to overt myeloma is usually associated with myeloma-defining events described in the acronym CRAB (hypercalcemia, renal insufficiency, anemia, and bone lesions). Herein we present a patient who presented with protein-losing enteropathy as the initial myeloma-defining event, secondary to intestinal lymphangiectasia caused by the immunoglobulin deposition and obstruction of intestinal lymphatics. After initiating treatment for myeloma, her symptoms rapidly resolved. We report this case to describe a rare myeloma-defining event caused by the raised paraprotein. This case illustrates the protean clinical manifestations in myeloma, and the importance of not restricting one's thinking to the traditional CRAB criteria, since delay in diagnosis of myeloma may result in end organ damage, complications, and increased morbidity.