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

Franck, Caspar; Venerito, Marino; Weigt, Jochen; Roessner, Albert; Malfertheiner, Peter

doi:10.1055/s-0043-120924

Cited by 9 publications

(8 citation statements)

References 19 publications

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“…When it manifests through high digestive bleeding, it is uncommon to present in massive bleeding [ 12 ]. In this context, it is more common for it to occur in the form of intermittent melena.…”

Section: Discussionmentioning

confidence: 99%

Pseudotumoral Amyloidosis Presentation With Upper Digestive Bleeding

Gomes¹,

Carlos²,

Kum³

et al. 2023

Cureus

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Amyloidosis is a condition related to the extracellular deposition of abnormal fibrillar proteins. Gastric involvement may present as a systemic or localized form of the disease. The endoscopic presentation can vary from nodular, ulcerated, or infiltrative lesions. Clinical manifestations are nonspecific, including hyporexia, nausea, vomiting, weight loss, epigastralgia, and abdominal discomfort. Thus, amyloidosis can clinically and endoscopically mimic other diseases, such as neoplasms, syphilis, tuberculosis, and Crohn's disease, requiring a high suspicion. When it manifests with gastrointestinal bleeding, it most commonly occurs as intermittent melena. This report aims to present an unusual case of a patient with upper gastrointestinal bleeding exteriorized through melena secondary to amyloidosis with gastric involvement.

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Section: Discussionmentioning

confidence: 99%

Pseudotumoral Amyloidosis Presentation With Upper Digestive Bleeding

Gomes¹,

Carlos²,

Kum³

et al. 2023

Cureus

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“…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

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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“…It may be difficult to differentiate symptoms of autonomic neuropathy of the bowel from gastrointestinal involvement. Deposits may be asymptomatic or cause abdominal bloating [47], gastroparesis [48, 49], gastrointestinal bleeding [50, 51], weight loss, malabsorption, and dyspepsia.…”

Section: Clinical Manifestationsmentioning

confidence: 99%

When to Suspect a Diagnosis of Amyloidosis

Vaxman

Gertz

2020

Acta Haematol

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Amyloidosis is a group of complex diseases caused by extracellular deposition of pathological insoluble fibrillary protein in organs and tissues and may result in severe organ dysfunction. Despite the etiological heterogeneity of systemic amyloidosis, the clinical manifestations of the different forms of amyloidosis largely overlap and depend upon the effected organ. The signs and symptoms that should raise suspicion for the potential diagnosis of amyloidosis are usually nonspecific; therefore, establishing the diagnosis is difficult, and early diagnosis requires clinical suspicion. Light chain (AL) amyloidosis may present with highly specific signs such as macroglossia and periorbital purpura, but these signs are insensitive. Amyloidosis is still underdiagnosed, even though treatments are now available and are effective in improving patient's survival and quality of life. Cardiac amyloidosis is the major determinant of survival, and the earlier it is detected the better the survival. All MGUS patients should be routinely screened for AL amyloidosis by a focused history and physical examination and routine assessment of urine albumin. The aim of this review is to provide clinicians with knowledge about the signs and symptoms that raise the suspicion of amyloidosis, bearing in mind the importance of early diagnosis of this disease.

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

Cited by 9 publications

References 19 publications

Pseudotumoral Amyloidosis Presentation With Upper Digestive Bleeding

Pseudotumoral Amyloidosis Presentation With Upper Digestive Bleeding

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

When to Suspect a Diagnosis of Amyloidosis

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