University of Missouri Kansas City School of Medicine Kansas City, Missouri
Introduction: See case report
Case Description/Methods: A 74-year-old male with coronary artery disease presented to the hospital with chest pain and shortness of breath and later was discovered to have melanotic stools and anemia with a hemoglobin of 8.9g/dL. An upper endoscopy demonstrated congested, mosaic patterned gastric mucosa with adherent clots, easy friability, and oozing (Figures 1A and 1B). This was initially thought to be secondary to portal hypertensive gastropathy, however laboratory work-up and imaging did not reveal any evidence of cirrhosis or portal hypertension. With recurrent bleeding, repeat endoscopy was performed with similar findings. Duodenal biopsies were then performed which revealed apple-green birefringence after congo red staining consistent with the diagnosis of amyloidosis later specified as alpha light chain (AL) amyloidosis by liquid chromatography (Figures 1C and 1D). Oncology was consulted and performed a bone marrow biopsy which was notable for a plasma cell dyscrasia with focal amyloid deposition and 4-5% plasma cells by CD138 immunostaining. The patient was started on Daratumumab, Cyclophosphamide, and Bortezomib however the patient only tolerated a short course before being readmitted for a recurrent upper gastrointestinal bleed and is being evaluated for an elective partial gastrectomy given his recurrence of symptoms.
The association of AL amyloidosis and a plasma cell dyscrasia is well known as studies show that around 80% of patients with AL amyloidosis have an underlying plasma cell line dysfunction.1 What was unique to this case was the initial presentation of AL amyloidosis and subsequent underlying plasma cell dyscrasia as isolated recurrent gastric hemorrhage which is a rare disease process.2 The diagnosis can be easily overlooked in patients who have not been diagnosed previously with amyloidosis or have an underlying plasma cell dyscrasia. Amyloidosis should also be considered in patients with unexplained persistent bleeding after cold biopsy or cold snare excision. This case serves as a reminder to keep a wide differential including systemic pathologies when initial workup is unrevealing.
