Srivastava Manjul
Internal Medicine, Northwest Medical Center, Tucson, USA.
Cureus. 2025 Jan 2;17(1):e76791. doi: 10.7759/cureus.76791. eCollection 2025 Jan.
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.
轻链型（AL）淀粉样变性所致自发性肝出血是一种罕见但严重的危及生命的疾病。在本病例中，一名64岁男性患者因急性右肩痛就诊于急诊室。他最近被诊断为2型糖尿病。他近期没有任何外伤、手术史，无肝炎、酗酒史，也没有慢性炎症或出血性疾病史。患者腹部计算机断层扫描（CT）显示急性包膜下血肿阳性。入院两天内他出现了急性肾病综合征。进一步检查显示单克隆λ轻链升高，骨髓活检确诊为AL淀粉样变性。该病例独特之处在于肝包膜下血肿是AL淀粉样变性的唯一表现。虽然系统性淀粉样变性中确实会发生淀粉样蛋白沉积，但患者在没有任何先前诊断的情况下出现自发性肝破裂极为罕见。认识到AL淀粉样变性的这种非典型表现对于及时干预和改善预后至关重要。熟悉肝内出血的罕见病因可能有助于降低与这种罕见疾病相关的高死亡率。肝出血的治疗包括积极输血和CT引导下肝动脉栓塞。环磷酰胺、硼替佐米和地塞米松（CyBorD）化疗可阻止淀粉样蛋白沉积。
