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

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

Öz

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.

Anahtar Kelimeler

• amiloidoz
• intestinal amiloidoz
• ileus
• ince bağırsak
• bağırsak kitlesi

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