AA amyloidosis presented with ileus by forming a mass in the small intestine

Year 2022, Volume: 7 Issue: 1, 21 - 23, 28.03.2022

Meryem İlkay Eren Karanis Ramazan Saygin Kerimoglu İlknur Küçükosmanoğlu Nermin Keni Begendi

https://doi.org/10.25000/acem.1050566

Abstract

Introduction:
Intestinal amyloidosis frequently encountered as a part of systemic amyloidosis, but rarely can be confined in the gastrointestinal tract.
Case report:
A 54-year-old male presented with the complaint of gas and stool discharge. Urgently segmental bowel resection was performed for ileus. Macroscopically nodular lesions, the largest at 7x3x0.7 cm in size were observed in the intestinal lumen. Microscopically; the accumulation of dense eosinophilic material that formed a mass in the submucosal area was noted. This material was positive with Crystal Violet, Congo Red and Amyloid A. Kappa and Lambda were negative.
No monoclonal gammopathy, increase in serum amyloid A levels, chronic inflammatory disease, infectious disease or malignancy was determined. The case was evaluated as "intestinal AA amyloidosis".
Discussion:
While AA amyloidosis was existent in our case, it comprised a mass lesion and caused intestinal obstruction. It is also exraordinary for AA amyloidosis to be confined in gastrointestinal tract.

Keywords

amyloidosis, intestinal amyloidosis, ileus, small intestine, intestinal mass

İnce bağırsakta kitle oluşturarak ileus ile kendini gösteren AA amiloidoz

Abstract

Giriş:
İntestinal amiloidoza sistemik amiloidozun bir parçası olarak sıklıkla rastlanır, ancak nadiren gastrointestinal kanalda sınırlı olabilir.
Olgu sunumu:
54 yaşında erkek hasta gaz ve gaita çıkaramama şikayeti ile başvurdu. İleus nedeniyle acil olarak segmental barsak rezeksiyonu yapıldı. Makroskopik olarak bağırsak lümeninde en büyüğü 7x3x0.7 cm boyutlarında nodüler lezyonlar gözlendi. Mikroskobik olarak; submukozal alanda kitle oluşturan yoğun eozinofilik materyal birikimi kaydedildi. Bu materyal Crystal Violet, Congo Red ve Amiloid A ile pozitifti. Kappa ve Lambda negatifti.
Monoklonal gamopati, serum amiloid A düzeylerinde artış, kronik inflamatuar hastalık, enfeksiyöz hastalık veya malignite saptanmadı. Olgu "intestinal AA amiloidoz" olarak değerlendirildi.
Tartışma:
Olgumuzda AA amiloidoz mevcut iken, kitle lezyonu oluşturmuş ve intestinal obstrüksiyona neden olmuştur. AA amiloidozun gastrointestinal kanalda sınırlı olması da olağandışıdır.

Keywords

amiloidoz, intestinal amiloidoz, ileus, ince bağırsak, bağırsak kitlesi

References

• Reference1- Iida T, Yamano H, Nakase H. Systemic amyloidosis with gastrointestinal involvement: Diagnosis from endoscopic and histological views. J Gastroen Hepatol 2018;33(3):583-590.
• Reference2- Petre S, Shah IA, Gilani N. gastrointestinal amyloidosis–clinical features, diagnosis and therapy. Aliment Pharm Ther 2008;27(11):1006-1016.
• Reference3- Rowe K, Pankow J, Nehme F, Salyers W. Gastrointestinal amyloidosis: review of the literature. Cureus 2017;9(5).
• Reference4- Gould M, Zarrin-Khameh N, Sellin J. Small bowel amyloidosis. Curr Gastroenterol Rep 2013;15(10):350.
• Reference5- Hu H, Huang D, Ji M, Zhang S. Multiple myeloma with primary amyloidosis presenting with digestive symptoms: A case report and literature review. Arab J Gastroenterol 2020;21:54-58
• Reference6- den Braber‐Ymker M, Heıjker S, Lammens M, Croockewıt S, Nagtegaal ID. Intestinal involvement in amyloidosis is a sequential process. Neurogastroent Motil 2018;30(12):e13469.
• Reference7- Bansal R, Syed U, Walfish J, Aron J, Walfish A. Small bowel amyloidosis. Curr Gastroenterol Rep 2018;20(3):11.
• Reference8- Schönland So, Hegenbart U, Bochtler T, Mangatter A, Hansberg M, Ho AD, et al. Immunohistochemistry in the classification of systemic forms of amyloidosis: a systematic investigation of 117 patients. Blood 2012;119(2):488-493.
• Reference9- Tada S, Iida M, Yao T, Kawakubo K, Yao T, Okada M, et al. Endoscopic features in amyloidosis of the small intestine: clinical and morphologic differences between chemical types of amyloid protein. Gastrointest Endosc 1994;40:45‐50.
• Reference10- Yamada M, Hatakeyama S, Tsukagoshı H. Gastrointestinal amyloid deposition in AL (primary or myeloma‐associated) and AA (secondary) amyloidosis: diagnostic value of gastric biopsy. Hum Pathol 1985;16:1206‐1211.
• Reference11- Fonnescu C, Giovinale M, Verrecchia E, De Socıio G, Cerquaglia C, Curigliano V. et al. Gastrointestinal amyloidosis: a case of chronic diarrhea. Eur Rev Med Pharmacol Sci 2009;13 suppl 1:45–50