Amyloidosis: an unusual cause of upper gastrointestinal bleeding

Siau, Keith; Elzubeir, Amera; Cooper, Sheldon C.; Iqbal, Tariq

doi:10.1136/bcr-2016-217653

Cited by 11 publications

(12 citation statements)

References 19 publications

(38 reference statements)

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“…Recent case reports describe UGIB in patients with known multiple myeloma or monoclonal gammopathy of unknown significance [16,17]. This case differs because the patient lacked a prior diagnosis and his presentation of recurrent UGIB led to the discovery of his underlying AL amyloidosis.…”

Section: Discussionmentioning

confidence: 97%

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

Chan

Carpentier

2021

BMC Gastroenterol

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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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Section: Discussionmentioning

confidence: 97%

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

Chan

Carpentier

2021

BMC Gastroenterol

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“…Data are lacking regarding the prevalence of lower gastrointestinal bleeding as a result of amyloidosis, resulting in limited evidence-based guidance regarding optimal evaluation and management. However, amongst patients with GI amyloidosis, between 25 and 45% present with gastrointestinal bleeding [1]. In cases of suspected GI amyloidosis, rectal biopsies should be considered, as the sensitivity is approximately 75 to 85 % compared with other sites [13].…”

Section: Discussionmentioning

confidence: 99%

“…Involvement of the gastrointestinal (GI) tract occurs in only about 3-8% of all cases of AA amyloidosis. Within the GI tract, amyloid deposits confirmed by histology are most often detected in the small intestine (50%), stomach (44%), colon (32%), esophagus (12%), and rectum (8%) [1].…”

Section: Introductionmentioning

confidence: 99%

Secondary Amyloidosis Presenting as Ischemic Proctitis

Hashmi

Munis

Hoff

et al. 2021

Case Reports in Gastrointestinal Medicine

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A 49-year-old man presented with abdominal pain and rectal bleeding for two days associated with a 50-pound unintentional weight loss. History was notable for hypertension, chronic kidney disease, obesity, gout, and acute cholecystitis status post cholecystectomy. Computed tomography (CT) of the abdomen and pelvis showed rectal wall thickening. Colonoscopy showed proctitis with superficial ulcerations. In the setting of renal insufficiency, malabsorption, and low-voltage QRS complexes on electrocardiogram (ECG), amyloidosis was considered in the differential diagnosis. Rectal and renal biopsies with subsequent retrospective staining of gallbladder tissue confirmed amyloid deposition. Gastrointestinal involvement of amyloidosis is relatively uncommon. Particularly, amyloid deposition in the gallbladder and rectum is very rare. The development of AA amyloidosis in our patient may have been related to gout, obesity, and the presence of a heterozygous complex variant for the MEFV (familial Mediterranean fever) gene. Awareness of this atypical presentation of amyloidosis is important, as additional staining of biopsy samples is necessary, and diagnosis allows for directed treatment.

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“…In such risk groups, periodic amyloidosis screening by determination of the serum NT-proBNP and urinary albumin concentration is recommended [9]. According to the experience described in a number of case reports, in patients with known MGUS and unspecific GI symptoms or GI bleeding, GI amyloidosis should be checked early by endoscopic biopsies [15,16].…”

Section: Discussionmentioning

confidence: 99%

Recurrent diffuse gastric bleeding as a leading symptom of gastrointestinal AL amyloidosis

et al. 2017

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Amyloidosis is a rare disease (incidence about 0.8/100 000) characterized by extracellular tissue deposition of fibrils composed of low molecular weight subunits of a variety of serum proteins. Clinical manifestations are largely determined by the type of precursor protein, the tissue distribution and the amount of amyloid deposition. Gastrointestinal (GI) manifestations of amyloidosis are even more uncommon (3 % of all amyloidosis patients). Symptoms of GI amyloidosis are nonspecific, heterogeneous, and include weight loss, GI bleeding, heartburn, early satiety, diarrhea and abdominal pain. The histopathological examination of biopsy specimens from the GI tract leads to the diagnosis.Herein we report the case of a 63-year-old woman with recurrent diffuse gastric bleeding. Endoscopic biopsies revealed distinct amyloid deposits in the mucosa of the stomach. Further histochemical assessment confirmed systemic light chain (AL) amyloidosis with clinically predominant gastrointestinal manifestation. An induction therapy with bortezomib and dexamethasone was initiated.Our report illustrates the importance of the multidisciplinary approach for diagnosis and management of AL amyloidosis. Current treatment of systemic AL amyloidosis is based on cytostatic targeting of immunoglobulin producing plasma cells. Therapeutic options are limited and highly toxic, making the development of novel treatment approaches an urgent need.

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Amyloidosis: an unusual cause of upper gastrointestinal bleeding

Cited by 11 publications

References 19 publications

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

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

Secondary Amyloidosis Presenting as Ischemic Proctitis

Recurrent diffuse gastric bleeding as a leading symptom of gastrointestinal AL amyloidosis

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