What is the most common presentation in patients with IPSID (immunoproliferative small intestinal disorder)?
High-Yield Explanation
Since diarrrhoea / steatorrhea is not there in the choice, the most common presentation is malabsorption. Immunoproliferative small intestinal disorder is primarily a disease of young adults. In early stage of the disease, there is diffuse infiltration of the mucosa and submucosa with B lymphocytes and plasma cells. As the disease progresses, the infiltrating cells develop histologically malignant characteristics, and mesenteric nodes become involved. Patients usually presents with anorexia, weight loss, diarrhea, steatorrhea, and abdominal pain. Lab investigation shows presence of a paraprotein consisting of the Fc poion of IgA, devoid of light chains, that migrates as a broad band on protein electrophoresis in the 2 and regions.