Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report

Abstract Background 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 d...

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Main Authors: Rachael Chan, Stephanie Carpentier
Format: Article
Language:English
Published: BMC 2021-07-01
Series:BMC Gastroenterology
Subjects:
Online Access:https://doi.org/10.1186/s12876-021-01882-7
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author Rachael Chan
Stephanie Carpentier
author_facet Rachael Chan
Stephanie Carpentier
author_sort Rachael Chan
collection DOAJ
description Abstract Background 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. Case presentation 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. Conclusions 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.
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spelling doaj.art-8aa0bf117ae64bd6bebbecfd3da37d032022-12-21T23:33:14ZengBMCBMC Gastroenterology1471-230X2021-07-012111410.1186/s12876-021-01882-7Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case reportRachael Chan0Stephanie Carpentier1Department of Medicine, Dalhousie University and Nova Scotia Health, QEII Health Sciences CentreDepartment of Medicine, Dalhousie University and Nova Scotia Health, QEII Health Sciences CentreAbstract Background 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. Case presentation 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. Conclusions 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.https://doi.org/10.1186/s12876-021-01882-7Upper GI bleedingAmyloidosisCase report
spellingShingle Rachael Chan
Stephanie Carpentier
Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
BMC Gastroenterology
Upper GI bleeding
Amyloidosis
Case report
title Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
title_full Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
title_fullStr Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
title_full_unstemmed Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
title_short Gastric amyloidosis presenting as acute upper gastrointestinal bleeding: a case report
title_sort gastric amyloidosis presenting as acute upper gastrointestinal bleeding a case report
topic Upper GI bleeding
Amyloidosis
Case report
url https://doi.org/10.1186/s12876-021-01882-7
work_keys_str_mv AT rachaelchan gastricamyloidosispresentingasacuteuppergastrointestinalbleedingacasereport
AT stephaniecarpentier gastricamyloidosispresentingasacuteuppergastrointestinalbleedingacasereport