Primary (AL) amyloidosis with gastrointestinal involvement

Madsen, Lone Galmstrup; Gimsing, Peter; Schiodt, Frank V.

doi:10.1080/00365520902783717

Cited by 41 publications

(38 citation statements)

References 16 publications

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“…For these patients, weight loss, diarrhea, and early satiety were among the most common symptoms reported, in keeping with prior reports. 10,11 These symptoms can also occur with autonomic neuropathy due to amyloidosis, and are not diagnostic of GI involvement in the absence of biopsy-proof.…”

Section: Discussionmentioning

confidence: 99%

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

et al. 2012

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Section: Discussionmentioning

confidence: 99%

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

et al. 2012

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“…Those presenting with GI manifestations often have indications for endoscopy such as weight loss, dyspepsia refractory to medical therapy or GI bleeding [26-27]. Any site can be affected, but the small bowel is most commonly affected [28].…”

Section: Reviewmentioning

confidence: 99%

Gastrointestinal Amyloidosis: Review of the Literature

Rowe¹,

Pankow²,

Nehme³

et al. 2017

Cureus

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Gastrointestinal amyloidosis (GIA), a protein deposition disorder, represents a complex common pathway that encompasses multiple etiologies and presentations. It represents a significant diagnostic and treatment challenge. The disease results from the deposition of insoluble extracellular protein fragments that have been rendered resistant to digestion. GIA can be acquired or genetic, and most commonly results from chronic inflammatory disorders (AA amyloidosis), hematologic malignancy (AL amyloidosis), and end-stage renal disease (Beta-2 amyloidosis). The deposition of these abnormal proteins interferes with gastrointestinal tract (GI) organ structure and function, most notably in the liver and small bowel. Presentation from GI involvement includes cirrhotic sequelae, abdominal pain, malabsorption, and GI bleeding. Diagnosis hinges on pathologic examination of affected tissue, with classic green birefringence under polarized light. Abdominal fat pad and rectal mucosal biopsy have been described as sites of higher sensitivity for diagnosis. Serum amyloid P scintigraphy is near 90% sensitive for diagnosis of AA amyloidosis. Patients should be considered for further evaluation to rule out additional organ involvement, notably cardiac and renal. Treatment hinges on an adequate suppression of the predisposing inflammatory disorder, or malignancy, followed by supportive therapy. Prognosis varies depending on the etiology of the disease, with the AL subtype showing worse outcomes, as well as those with hepatic involvement.

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“…A retrospective review of data collected from patients with all types of amyloidosis showed that only 3.2% had biopsy-proven amyloid involvement of the gastrointestinal tract [5]. The mean age of presentation for primary systemic light-chain amyloidosis is usually in the sixth to seventh decade of life, as evidenced by multiple retrospective studies [5, 6, 7, 8]. Gastrointestinal involvement in light-chain amyloidosis is defined by the presence of gastrointestinal symptoms with direct biopsy verification of amyloid deposition.…”

Section: Introductionmentioning

confidence: 99%

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

Gaduputi

Badipatla

Patel

et al. 2013

Case Rep Gastroenterol

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We report this case of a 42-year-old woman who presented with a debilitating illness manifested by intractable nausea, vomiting, diarrhea and unchecked weight loss. The patient had multisystem involvement that presented as anemia, abnormal liver function tests and progressively deteriorating renal function necessitating dialysis. She was found to be profoundly hypoalbuminemic secondary to malabsorptive and protein-losing enteropathy in tandem with nephrotic range proteinuria. Intolerance to enteral feeding led the patient to be dependent on parenteral nutrition. Serum immunofixation revealed IgG lambda monoclonal protein. The patient underwent endoscopic evaluation with biopsies taken from the gastrointestinal tract that confirmed the diagnosis of primary systemic light-chain amyloidosis. A subsequent bone marrow biopsy revealed normocellular bone marrow with deposition of amyloid. The patient was not considered for autologous stem cell transplantation as the outcomes in patients with multisystem involvement are often poor, with a high mortality risk. Diffuse primary systemic light-chain amyloidosis involving the gastrointestinal tract is a rare entity and is to be considered among differentials in patients presenting with unexplained malabsorptive symptoms.

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Primary (AL) amyloidosis with gastrointestinal involvement

Abstract: Most patients with gastrointestinal AL amyloidosis experience weight loss and all have signs of malabsorption. Despite treatment the prognosis is grave.

Cited by 41 publications

References 16 publications

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

Amyloidosis of the gastrointestinal tract: a 13-year, single-center, referral experience

Gastrointestinal Amyloidosis: Review of the Literature

Primary Systemic Amyloidosis with Extensive Gastrointestinal Involvement

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