Spontaneous Hepatic Hemorrhage as a Rare Presentation of Amyloid Light Chain (AL) Amyloidosis: A Case Report.

Spontaneous hepatic hemorrhage due to amyloidosis light chain (AL) is a rare but serious life-threatening condition. In this case, a 64-year-old male patient presented to the emergency room with acute right shoulder pain. He was recently diagnosed with type 2 diabetes. He did not have any recent trauma, procedures, history of hepatitis, alcoholism, or chronic inflammatory or bleeding disorders. The patient's computed tomography (CT) scan of the abdomen was positive for acute subcapular hematoma. He developed acute nephrotic syndrome within two days of admission. Further workup showed elevated monoclonal lambda light chains and confirmed AL amyloidosis on bone marrow biopsy. This case is unique because subcapsular hepatic hematoma was the only manifestation of AL amyloidosis. While amyloid deposition does occur in systemic amyloidosis, it is exceptionally rare for patients to present with spontaneous hepatic rupture without any prior diagnosis. Recognizing this atypical presentation of AL amyloidosis is crucial for timely intervention and improved outcomes. Familiarity with the rare etiology of intrahepatic hemorrhage may help to decrease the high mortality rate associated with this rare disease. Treatment for hepatic hemorrhage consists of aggressive blood transfusion and CT-guided hepatic artery embolization. Chemotherapy with cyclophosphamide, bortezomib, and dexamethasone (CyBorD) can halt amyloid deposition.