Shiroshita Akihiro, Uegami Wataru, Otsuki Ayumu, Nakashima Kei, Sunagawa Keishin, Fukuoka Junya, Kami Shota, Saito Ayumi, Aoshima Masahiro
Department of Pulmonology, Kameda Medical Center, 929 Higashi-cho, Kamogawa 296-8602, Chiba Prefecture, Japan.
Department of Anatomic Pathology, Kameda Medical Center, 929 Higashi-cho, Kamogawa 296-8602, Chiba Prefecture, Japan.
Oxf Med Case Reports. 2019 Aug 1;2019(8). doi: 10.1093/omcr/omz074.
We report the case of a 61-year-old woman with Kartagener syndrome who presented with a 3-month history of chronic watery diarrhoea and severe hypoalbuminaemia. Histopathological examination of duodenum and large intestine biopsies showed amyloid A (AA) amyloid deposition. Scintigraphy and alpha-1 anti-trypsin clearance evaluations revealed protein-losing gastroenteropathy. Computed tomography with contrast and positron emission tomography showed a pelvic mass with multiple para-aortic lymph node enlargement. We suspected protein-losing gastroenteropathy secondary to AA amyloid produced related to malignant tumours. Following tumour resection, histopathological examination of the lesion revealed undifferentiated carcinoma of unknown origin. Postoperatively, the patient's nutritional condition improved. There has been no recurrence of protein-losing gastroenteropathy 6 months postoperatively. This is the first report of protein-losing gastroenteropathy and AA amyloidosis secondary to undifferentiated carcinoma. Early recognition and intervention could increase the likelihood of amyloidosis remission.
我们报告了一例61岁患有卡塔格内综合征的女性患者，她有3个月的慢性水样腹泻病史和严重低白蛋白血症。十二指肠和大肠活检的组织病理学检查显示有淀粉样蛋白A（AA）淀粉样沉积。闪烁扫描和α-1抗胰蛋白酶清除率评估显示为蛋白丢失性胃肠病。增强计算机断层扫描和正电子发射断层扫描显示盆腔肿块伴多发腹主动脉旁淋巴结肿大。我们怀疑是与恶性肿瘤相关的AA淀粉样蛋白产生继发的蛋白丢失性胃肠病。肿瘤切除后，病变的组织病理学检查显示为来源不明的未分化癌。术后，患者的营养状况有所改善。术后6个月蛋白丢失性胃肠病未复发。这是关于未分化癌继发蛋白丢失性胃肠病和AA淀粉样变性的首例报告。早期识别和干预可能会增加淀粉样变性缓解的可能性。
