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Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
BMC Gastroenterology ( IF 2.4 ) Pub Date : 2021-07-29 , DOI: 10.1186/s12876-021-01882-7
Rachael Chan 1 , Stephanie Carpentier 1, 2
Affiliation  

Amyloidosis is characterized by extracellular tissue deposition of fibrils, composed of insoluble low-molecular-weight protein subunits. The type, location, and extent of fibril deposition generates variable clinical manifestations. Gastrointestinal (GI) bleeding due to amyloid deposition is infrequent. Previous literature describes upper GI bleeding (UGIB) in patients with known amyloid disease. Here, we describe a case of recurrent UGIB that ultimately led to a diagnosis of GI amyloidosis and multiple myeloma in a patient with no history of either. A 76-year-old male presented to the emergency department with frank hematemesis, melena, and a decreased level of consciousness. Management required intensive care unit (ICU) admission with transfusion, intubation, and hemodynamic support. Upper endoscopy revealed gastritis with erosions and nodularity in the gastric cardia and antrum. Hemostasis of a suspected bleeding fundic varix could not be achieved. Subsequently, the patient underwent computerized tomography (CT) angiography and an interventional radiologist completed embolization of the left gastric artery to address potentially life-threatening bleeding. Complications included development of bilateral pleural effusions and subsegmental pulmonary emboli. Pleural fluid was negative for malignancy. He was transferred to a peripheral hospital for continued care and rehabilitation. Unfortunately, he began re-bleeding and was transferred back to our tertiary center, requiring re-admission to the ICU and repeat endoscopy. Repeat biopsy of the gastric cardial nodularity was reported as active chronic gastritis and ulceration. However, based on the unusual endoscopic appearance, clinical suspicion for malignancy remained high. He exhibited symptoms of congestive heart failure following standard resuscitation. Transthoracic echocardiogram (TTE) demonstrated a reduced ejection fraction of 35–40% and a strain pattern with apical sparing. Following discussions between the treating gastroenterologist, consulting cardiologist, and pathologist, Congo Red staining was performed, revealing submucosal amyloid deposits. Hematology was consulted and investigations led to diagnosis of multiple myeloma (MM) and immunoglobulin light-chain (AL) amyloidosis. The patient was treated for MM for four months prior to cessation of therapy due to functional and cognitive decline. GI amyloidosis can present with various non-specific clinical symptoms and endoscopic findings, rendering diagnosis a challenge. This case illustrates GI amyloidosis as a potential—albeit rare—etiology of UGIB.

中文翻译:

以急性上消化道出血为表现的胃淀粉样变1例报告

淀粉样变性的特征在于原纤维的细胞外组织沉积,原纤维由不溶性低分子量蛋白质亚基组成。原纤维沉积的类型、位置和程度会产生不同的临床表现。由于淀粉样蛋白沉积引起的胃肠 (GI) 出血很少见。以前的文献描述了已知淀粉样疾病患者的上消化道出血 (UGIB)。在这里,我们描述了一个复发性 UGIB 病例,该病例最终导致一名没有任何病史的患者被诊断为 GI 淀粉样变性和多发性骨髓瘤。一名 76 岁男性因明显的呕血、黑便和意识水平下降到急诊室就诊。管理需要重症监护病房 (ICU) 入院,并进行输血、插管和血流动力学支持。上消化道内镜检查显示胃炎伴胃贲门和胃窦糜烂和结节。无法实现怀疑出血的胃底静脉曲张的止血。随后,患者接受了计算机断层扫描 (CT) 血管造影术,介入放射科医生完成了胃左动脉栓塞术,以解决可能危及生命的出血。并发症包括发生双侧胸腔积液和亚段肺栓塞。胸腔积液为恶性肿瘤阴性。他被转移到周边医院继续护理和康复。不幸的是,他开始再次出血并被转回我们的三级中心,需要重新入住 ICU 并再次进行内窥镜检查。胃贲门结节的重复活检报告为活动性慢性胃炎和溃疡。然而,基于不寻常的内窥镜外观,临床对恶性肿瘤的怀疑仍然很高。他在标准复苏后表现出充血性心力衰竭的症状。经胸超声心动图 (TTE) 显示射血分数降低了 35-40%,并且出现了心尖保留的应变模式。在治疗胃肠病学家、咨询心脏病学家和病理学家之间讨论后,进行了刚果红染色,揭示了黏膜下淀粉样蛋白沉积物。咨询了血液学,并通过调查诊断出多发性骨髓瘤 (MM) 和免疫球蛋白轻链 (AL) 淀粉样变性。由于功能和认知能力下降,患者在停止治疗前接受了四个月的 MM 治疗。胃肠道淀粉样变性可表现为各种非特异性临床症状和内镜检查结果,使诊断成为一项挑战。该病例说明 GI 淀粉样变性是 UGIB 的潜在病因(尽管很少见)。
更新日期:2021-07-29
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