A Case of Amyloid Light-Chain Amyloidosis Presenting With Submucosal Hematoma and Bleeding in the Upper Gastrointestinal Tract

Tanaka, Yukari; Hosotani, Kazuya; Fukushima, Masashi

doi:10.1016/j.cgh.2019.09.046

Cited by 5 publications

(4 citation statements)

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“…Распространенное отложение амилоида в ЖКТ сопровождается поражением печени, развитием желудочно-кишечных кровотечений (ЖКК) и псевдоопухолей (рис. 7), мальабсорбции, нарушением моторики ЖКТ, формированием протеин-теряющей гастропатии [18,21,22].…”

Section: гастроинтестинальные проявления Al-амилоидозаunclassified

“…NTproBNP -N-концевой пропептид мозгового натрийуретического гормона, BNP -мозговой натрийуретический гормон, ЩФ -щелочная фосфатаза, ГГТП -гамма-глутамил транспептидаза, МРТ -магниторезонансная томография кровотечения с локализацией в толстом кишечнике, желудке [23]. Известны случаи ишемии кишечника [18], острого спонтанного массивного ЖКК из верхних [22,24,25,26] и нижних отделов ЖКТ [27], а также рецидивирующего желудочного кровотечения [28]. В ряде случаев массивное кровотечение сочетается с клиникой псевдообструкции кишечника за счет наличия амилоидных полипов, дивертикулов [21].…”

Section: примечаниеunclassified

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Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist

Губергріц¹,

Byelyayeva²,

Mozhyna³

et al. 2022

jour

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The purpose of the review is to present an analysis of modern literature data on the pathogenesis, diagnosis and therapy of primary systemic amyloidosis of immunoglobulin light chains (AL-amyloidosis), to reveal the features of gastroenterological manifestations of the disease. AL-amyloidosis is caused by overproduction of immunoglobulin light chains by a clone of plasma cells located in the bone marrow, followed by the formation of amyloid.Deposition of amyloid in target organs (heart, kidneys, liver, gastrointestinal tract, peripheral and autonomic nervous systems, soft tissues) is accompanied by direct and indirect cytotoxic effects on organs and tissues. Gastrointestinal manifestations of AL-amyloidosis include liver damage, gastrointestinal bleeding, pseudo-obstruction of the small intestine and colon, the appearance of polyp-, diverticul-, tumor-like formations, malabsorption, impaired motility of the gastrointestinal tract, protein-losing gastropathy. Liver damage in patients with AL-amyloidosis, as a rule, is accompanied by minimal clinical manifestations, an increase of the level of alkaline phosphatase without any other reasons for this change. A detailed analysis of the Mayo Clinic’s practical guidelines for the diagnosis and treatment of AL-amyloidosis is presented. Diagnosis of gastrointestinal AL-amyloidosis is based on histomicroscopic analysis of biopsies of target organs with Congo red and subsequent examination in polarized light; mass spectrometry is the gold standard of diagnostic. Modern pharmacotherapy of AL-amyloidosis includes a combination of high-dose chemotherapy with monoclonal antibodies, proteasome inhibitors, cytostatics, hormones, as well as performing autologous stem cell transplantation. Correction of gastroenterological manifestations of the disease is based on the symptomatic therapy. Life expectancy of patients with AL-amyloidosis is determined by several prognostic models; the Boston University model, based on the definition of two markers, is most convenient for clinical use.

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Section: гастроинтестинальные проявления Al-амилоидозаunclassified

Section: примечаниеunclassified

Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist

Губергріц¹,

Byelyayeva²,

Mozhyna³

et al. 2022

jour

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“…The most frequently reported bleedings are ecchymosis and purpura; bleedings from the gastrointestinal tract and the renal tract are also common 38 , 39 , 40 , 41 ( Table 3 ). Amyloid deposits can be found in digestive and mucous membranes.…”

Section: Amyloidosis and Bleeding Riskmentioning

confidence: 99%

Thromboembolism and bleeding in systemic amyloidosis: a review

et al. 2021

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The assessment of both thromboembolic and haemorrhagic risks and their management in systemic amyloidosis have been poorly emphasized so far. This narrative review summarizes main evidence from literature with clinical perspective. The rate of thromboembolic events is as high as 5–10% amyloidosis patients, at least in patients with cardiac involvement, with deleterious impact on prognosis. The most known pro‐thrombotic factors are heart failure, atrial fibrillation, and atrial myopathy. Atrial fibrillation could occur in 20% to 75% of systemic amyloidosis patients. Cardiac thrombi are frequently observed in patients, particularly in immunoglobulin light chains (AL) amyloidosis, up to 30%, and it is advised to look for them systematically before cardioversion. In AL amyloidosis, nephrotic syndrome and the use of immunomodulatory drugs also favour thrombosis. On the other hand, the bleeding risk increases because of frequent amyloid digestive involvement as well as factor X deficiency, renal failure, and increased risk of dysautonomia‐related fall.

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“…Gastrointestinal manifestations of amyloidosis include dysmotility, malabsorption, and bleeding (2). Previous case reports described adult amyloidosis patients with gastrointestinal involvement presenting with hematemesis (3,4). We report a rare pediatric case of systemic AL amyloidosis presenting with upper gastrointestinal bleeding.…”

Section: Figurementioning

confidence: 99%

A Rare Case of Pediatric Amyloidosis Presenting as Upper Gastrointestinal Bleeding

Dike

Pereira

Kaufman

et al. 2021

J. pediatr. gastroenterol. nutr.

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A 15-year old Hispanic male with asthma presented with 1-week of hematemesis, melena, and 1month 10 lb weight loss. His hemoglobin was 7.7 g/dL, and he received a red blood cell transfusion and intravenous acid blockade medication. His white blood cells, platelets, electrolytes, albumin, and international normalized ratio were normal. Initial endoscopic features showed focal ecchymoses, diffuse mucosal nodularity, and oozing; however, specimen pathology lacked significant abnormalities. Abdominal CT and PET scan demonstrated gastric wall thickening, nodularity, and hypermetabolic activity, respectively. Oncology recommended full-thickness biopsies to better evaluate for malignancy. Repeat endoscopy with surgical laparoscopic mucosal resection and wedge biopsies revealed a markedly thickened muscular wall and chronic inflammation (Fig. 1). Workup for malignancies, infections, rheumatologic or autoinflammatory disorders was negative. Subsequent esophagogastroduodenoscopy and lacrimal duct specimens were obtained to perform additional diagnostic tissue staining and were Congo red positive with birefringence via polarized light, consistent with amyloid deposition (Fig. 2). LC-MS/MS confirmed AL (lambda)-type amyloid deposition. Pulmonary function tests and echocardiogram were normal.Amyloidosis is a group of hereditary or acquired diseases marked by systemic or localized amyloid deposition leading to progressive organ dysfunction (1). An accumulation of abnormal proteins occurs in immunoglobulin light chain (AL) amyloidosis and aging-associated amyloidosis (1,2). Conversely, an excess of normal proteins occurs in systemic (AA) amyloidosis or ß2-microglobulin amyloidosis (1). Gastrointestinal manifestations of amyloidosis include dysmotility, malabsorption, and bleeding (2). Previous case reports described adult amyloidosis patients with gastrointestinal involvement presenting with hematemesis (3,4). We report a rare pediatric case of systemic AL amyloidosis presenting with upper gastrointestinal bleeding.

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A Case of Amyloid Light-Chain Amyloidosis Presenting With Submucosal Hematoma and Bleeding in the Upper Gastrointestinal Tract

Cited by 5 publications

References 0 publications

Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist

Modern concepts of primary systemic AL-amyloidosis: the viewpoint of a gastroenterologist

Thromboembolism and bleeding in systemic amyloidosis: a review

A Rare Case of Pediatric Amyloidosis Presenting as Upper Gastrointestinal Bleeding

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