A Rare Case of Light Chain Amyloidosis of Gastrointestinal Tract

A Rare Case of Light Chain Amyloidosis of the Gastrointestinal Tract.

• Kathrin Dvir
• Gliceida M Galarza-Fortuna
• Anna Willet
• Christopher Febres-Aldana
• Nathaly Cortez
• Samuel Rapaka
• Andre Coombs
• Robert Goldberg
• Michael Schwartz
• Kfir Ben-David

抄録

65歳のヒスパニック系女性が1年間の食欲不振，吐き気，早期満腹感，胃部不快感，20kgの体重減少を呈した．CT（コンピュータ断層撮影）では肝実質に不均一性が認められた．上部内視鏡検査では小胃湾曲部切欠部に真菌性の大きな浸潤性腫瘤を認め，胃腫瘍の疑いが強かったが，初期生検では不明瞭であったため，腹腔鏡検査を行った．術中の胃腫瘤および肝実質の生検を繰り返したところ、アミロイド沈着と一致するびまん性のヒアリン化したストローマが認められ、骨髄生検で原発性軽鎖（AL）アミロイドーシスの診断が確定した。

A 65-year-old Hispanic female presented with a one-year history of anorexia, nausea, early satiety, epigastric discomfort, and a 20 kg weight loss. Computed tomography (CT) demonstrated heterogeneous liver parenchyma. Upper endoscopy revealed large, fungating, infiltrative mass at the lesser gastric curvature incisura, highly suspicious of gastric tumor; however, initial biopsy of the gastric mass was equivocal and an exploratory laparoscopy was performed. Repeated intraoperative biopsies of the gastric mass and of liver parenchyma demonstrated diffuse hyalinized stroma consistent with amyloid deposition, and a bone marrow biopsy confirmed the diagnosis of primary light chain (AL) amyloidosis.

Copyright © 2020 Kathrin Dvir et al.