P1851 - Age Is Just a Number: A Rare Case of Primary Hepatic Amyloidosis in a Young Male

Nabeel Akhtar, MD¹, Monica Zukowski, BS², Adam Sturts, DO¹, Alison Faust, MD^2
1Penn State University Milton S. Hershey Medical Center, Hershey, PA; ²Penn State University Milton S. Hershey Medical Center and College of Medicine, Hershey, PA

Introduction: Hepatic amyloidosis can present as primary or, more commonly, secondary amyloidosis usually in the older population. Here, we present a rare case of primary hepatic amyloidosis in a previously, young and healthy male.

Case Description/Methods: A 37 year-old male presented as an outside hospital transfer with a 1-week history of abdominal pain and hematochezia and a 6-month history of fatigue, weight loss, ecchymosis, and lower extremity edema. Physical exam demonstrated hepatosplenomegaly, abdominal distension and ascites. Labs noted platelet count 465K/uL, white count 12K/uL, hemoglobin 14g/dL, INR 1.2, ALT 28unit/L, AST 46unit/L, alkaline phosphatase 829unit/L, total bilirubin 0.8mg/dL. Imaging showed hepatosplenomegaly, ascites and pancolitis. His C. difficile toxin genes were positive and he was treated for C. difficile infection without improvement in his diarrhea. A flexible sigmoidoscopy was negative for endoscopic features of inflammatory bowel disease and cultures were negative for other microorganisms. Biopsies were deferred due to concern for von Willebrand’s disease and bleeding risk.

A serologic workup for liver disease was negative for viral, autoimmune, and genetic etiologies. His urine studies supported nephrotic range proteinuria (4.6g/24h). He ultimately underwent renal biopsy that showed amyloid deposition. The monotypic staining for lambda light chains resulted in AL amyloidosis. Free lambda light chains were seen on urine immunofixation. A bone marrow biopsy revealed a plasma cell myeloma.

He was started on CyBorD induction therapy and Daratumumab. While he achieved hematologic remission, he developed worsening liver failure with encephalopathy, impaired coagulation factors, and progressive ascites consistent with portal hypertension – cytology was negative for malignancy. He was ultimately transferred to a center that would consider liver transplantation.

Discussion: We demonstrate a case of AL amyloidosis with plasma cell myeloma in a patient that is much younger than typically seen and with a more aggressive course than what is typically seen. AL amyloidosis (light chain associated) is associated with plasma cell dyscrasia. Treatment options include CyBorD and Daratumumab, not known to worsen liver disease. Hepatic involvement is seen in 62-90% of AL amyloidosis but clinical manifestations are usually mild, commonly hepatomegaly and elevated alkaline phosphatase. Jaundice, portal hypertension, and liver failure are rare.


Disclosures:
Nabeel Akhtar indicated no relevant financial relationships.
Monica Zukowski indicated no relevant financial relationships.
Adam Sturts indicated no relevant financial relationships.
Alison Faust indicated no relevant financial relationships.


Nabeel Akhtar, MD¹, Monica Zukowski, BS², Adam Sturts, DO¹, Alison Faust, MD². P1851 - Age Is Just a Number: A Rare Case of Primary Hepatic Amyloidosis in a Young Male, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.