Clinical Recognition of AL Type Amyloidosis of the Luminal Gastrointestinal Tract

James, Dustin G.; Zuckerman, Gary R.; Sayuk, Gregory S.; Wang, Hanlin L.; Prakash, Chandra

doi:10.1016/j.cgh.2007.02.038

Cited by 72 publications

(61 citation statements)

References 58 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Another retrospective study that included 231 Chinese AL patients did not describe GI hemorrhage as a representative symptom [7]. Furthermore, 35 % (6/17) of patients with GI AL amyloidosis and GI amyloid deposition showed submucosal hematomas during endoscopic examinations [8]. Accordingly, GI bleeding is thought to be a rare, but specific primary symptom of systemic AL amyloidosis.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom

Kon¹,

Nakagawa²,

Yoshikawa³

et al. 2016

Clin J Gastroenterol

View full text Add to dashboard Cite

Immunoglobulin light-chain (AL) amyloidosis is characterized by the deposition of insoluble fibrils composed of immunoglobulin light chains secreted by monoclonal plasma cells. Given the recent advances in the therapy of AL amyloidosis, it is important to diagnose this disease as early as possible. Herein, we describe the case of a 62-year-old man with hepatitis C virus (HCV)-related cirrhosis presenting with hematochezia. Colonoscopy showed multiple submucosal hematomas within the region ranging from the transverse colon to the sigmoid colon. Kappa immunoglobulin light-chain amyloid deposition was also detected. Bone marrow examination revealed a monoclonal abnormal plasma cell population. Thus, the patient was diagnosed with systemic immunoglobulin light-chain amyloidosis. The hematochezia was conservatively managed. However, because of liver failure caused by liver cirrhosis, the patient developed massive pleural effusion and died of respiratory failure. Postmortem examination revealed amyloid deposition in the esophagus, stomach, duodenum, ileum, descending colon, pancreas, heart, and lung. In these organs, amyloid deposition was limited to the vascular wall. We concluded that AL amyloidosis can present hematochezia arising from submucosal hematoma in the large colon before other systemic symptoms appear.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The formation of GI submucosal hematomas in cases of AL amyloidosis may be attributed to two possible mechanisms, namely acquired factor X deficiency [10][11][12] and vascular fragility [8,13,14]. In 154 patients with AL amyloidosis, subnormal factor X activity was found in 22 cases (14 %) [10].…”

Section: Discussionmentioning

confidence: 99%

Systemic immunoglobulin light-chain amyloidosis presenting hematochezia as the initial symptom

Kon¹,

Nakagawa²,

Yoshikawa³

et al. 2016

Clin J Gastroenterol

View full text Add to dashboard Cite

show abstract

“…L'examen endoscopique peut être normal dans 75 % des cas [9,20,21]. Ailleurs, il peut montrer des aspects variables tels que de fines granulations muqueuses, des érosions, des ulcérations, un épaississement des plis muqueux des héma-tomes sous-muqueux, des polypes pouvant être le résultat de dépôts intenses de protéine AL dans des régions circonscrites et d'une fragilité muqueuse [1,20] (Fig.…”

Section: Discussionunclassified

“…Le pronostic des patients atteints d'amylose digestive est péjoratif avec une médiane de survie de 12 mois [9,21]. Au moins 16 % des sujets décèdent de complications de l'amylose dans le mois suivant le diagnostic (complications hémor-ragiques, dénutrition sévère liée à la malabsorption) [20].…”

Section: Discussionunclassified

Amylose gastroduodénale: cause inhabituelle d’hémorragie digestive massive

Amouri¹,

Chtourou

Mnif

et al. 2011

Acta Endosc

View full text Add to dashboard Cite

L'amylose est caractérisée par le dépôt extracellulaire d'un matériel amyloïde dans divers tissus et organes. L'atteinte digestive chez les patients porteurs d'une amylose systémique est quasi constante, mais souvent asymptomatique. Une hémorragie digestive sévère est rare, mais elle peut être fatale. Nous rapportons l'observation d'un homme de 56 ans ayant une amylose gastroduodénale isolée, révélée par une hémorragie digestive grave. À travers ce cas et une revue de la littérature, nous insistons sur les difficultés de prise en charge et la gravité de cette localisation et de ses complications rares. Pour citer cette revue : Acta Endosc. 41 (2011). Mots clés Amylose · Myélome · HémorragieAbstract Amyloidosis is characterized by the extracellular deposition of amyloid protein in various organs. Gastrointestinal involvement in amyloidosis is common, but is often asymptomatic. Severe gastrointestinal haemorrhage is rare but can be fatal in some cases. We report the case of a 56-year-old man who presented with massive haematochezia revealing an AL amyloidosis. The amyloidosis was apparently limited to the digestive tract. Through this case and a review of the literature, we emphasize the therapeutic difficulties and the severity of this rare localization. To cite this journal: Acta Endosc. 41 (2011).

show abstract

“…The clinical characteristics such as the association with food and talking 11 provided a strong clue to the aetiology which was confirmed by objective testing. Gastro-oesophageal complications of amyloidosis are common 12 and it is perhaps only with the growing realisation that cough is a frequent manifestation of gastrointestinal (GI) pathology that a link was made. The mechanism of oesophageal dysfunction is obscure since amyloid deposition was confined to the vessels and there was no primary oesophageal smooth muscle infiltration.…”

mentioning

confidence: 99%