Hackensack University Medical Center Hackensack, NJ, United States
Davood K. Hosseini, MD1, Kristen Delcalzo, BA2, Antik Patel, BA2, Raul Rodriguez, MD3, Nilesh B. Shukla, MD4 1Hackensack University Medical Center, Hackensack, NJ; 2St. George's University School of Medicine, Hackensack, NJ; 3Rutgers New Jersey Medical School, Hackensack, NJ; 4The Valley Hospital, Glen Rock, NJ
Introduction: The most common form, systemic amyloidosis, is characterized by production of amyloidogenic precursor proteins at a site, remote from the organs of amyloid deposition.
Case Description/Methods: A 65-year old male with a 25 year history of chronic alcoholism, presented to the emergency department for a 2 week history of non-radiating right upper quadrant abdominal pain associated with pruritus, nausea, coffee ground emesis, and clay colored stools. The exam was notable for icteric sclera, right upper quadrant abdominal tenderness, ascites, and hepatomegaly. Initial work up revealed new onset unexplained elevated liver enzyme.
Abdominal Ultrasound was significant for hepatomegaly with perihepatic ascites. The CT scan revealed diffuse liver cirrhosis, periportal lymphadenopathy, and stigmata of portal hypertension including hepatosplenomegaly, intra-abdominal ascites, and varices. Esophagogastroduodenoscopy with endoscopic ultrasound was performed, which showed gastritis, and enlarged porta hepatis, which was ultimately biopsied and sent for cytology and pathology. Given the unexplained elevation of the liver enzymes, and liver biopsy revealed extracellular amyloid deposition in peri-sinusoidal spaces consistent with amyloidosis. Transesophageal echocardiogram which raised suspicion for cardiac involvement with amyloid deposit. Immunoglobulin levels were subsequently investigated and showed elevated IgG antibodies. A K/L free ratio was elevated to 94.7. A myeloma flow cytometry exhibited an IgG kappa monoclonal-gammopathy. Finally, a CT-guided bone marrow biopsy from the iliac crest was obtained, confirming an IgG kappa multiple myeloma with 80% proportion of plasma cells (DS 2A, ISS 3). The patient was started on steroids, and chemotherapy with daratumumab, however his condition was complicated by septic shock, which led to an admission in the ICU followed by endotracheal intubation and multi-organ failure and eventual palliative care.
Discussion: Localized GI and hepatic amyloidosis have rarely been described in the literature. This condition poses great diagnostic challenges and warrants extensive work up before definitive diagnosis. Our case highlights the importance of clinical suspicion of GI amyloidosis in patients with constitutional symptoms including fatigue, weight loss, and unexplained liver disease. Once amyloidosis is considered, the diagnosis can be obtained by tissue biopsy from either the GI tract or subcutaneous adipose tissue.
Figure: CT scan (A-B) showing hepatosplenomegaly, and peri-splenic varices (arrowheads). Pathologic findings (C-G) of AL type amyloidosis of the luminal gastrointestinal tract. C. Histologic examination (Hematoxylin-Eosin staining) of a gastric antral type mucosa with chronic inactive gastritis and interstitial deposition of pink amorphous material (white asterisk). Histologic examination of liver with amorphous material deposition: Hematoxylin-Eosin staining (black asterisk in D-E), and crystal violet staining (G). F. Vascular wall are almost always involved first in the systemic amyloidosis (Hematoxylin-Eosin staining).
Disclosures: Davood Hosseini indicated no relevant financial relationships. Kristen Delcalzo indicated no relevant financial relationships. Antik Patel indicated no relevant financial relationships. Raul Rodriguez indicated no relevant financial relationships. Nilesh Shukla indicated no relevant financial relationships.
Davood K. Hosseini, MD1, Kristen Delcalzo, BA2, Antik Patel, BA2, Raul Rodriguez, MD3, Nilesh B. Shukla, MD4. P1894 - A Rare Cause of Liver Cirrhosis and Gastritis: Primary Gastrointestinal Amyloidosis in a Patient With Multiple Myeloma, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.