Primary amyloidosis with initial gastrointestinal manifestation. A case report

Abstract

Introduction. Amyloidosis is a rare disease associated with extracellular accumulation of abnormal protein – amyloid in various organs and systems. This disease can be either acquired or hereditary, systemic or localized. At its core, it represents a grown, tumor-like neoplastic clone of the plasma cells in the bone marrow. Gastrointestinal amyloidosis is manifested by symptoms, such as diarrhea, steatorrhea, constipation, and very rarely – hemorrhages and perforations of the colon. Case presentation. We present a case of primary intestinal amyloidosis with recurrent hematochezia and abdominal pain in a 61-year-old woman. Colonoscopy revealed polyposis of the whole colon and a total colectomy was performed, followed by morphological and paraclinical examinations. Histologically, amyloid deposition, positive for Congo red, was found in the walls of the submucosal blood vessels and in the smooth muscle cells of the muscular layers. The laboratory tests indicated anemia, high erythrocyte sedimentation rate, and Bence-Jones proteins in urine. Conclusions. Our case is a demonstration of primary amyloidosis with intestinal localization that should be taken into consideration in the presence of recurrent hematochezia.

Keywords

• amyloid
• colon
• hematochezia
• histopathology