A case of primary systemic amyloidosis with amyloid deposits in the duodenum and bone marrow aspirate: A rare finding

IF 0.1 Q4 HEMATOLOGY Iraqi Journal of Hematology Pub Date : 2023-01-01 DOI:10.4103/ijh.ijh_15_23
M. Momin, A. Ingle, R. Reddy, G. Krishna Reddy
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Abstract

Amyloidosis is a group of diseases characterized by extracellular abnormal proteinaceous material (amyloid) deposition in various organs. As amyloid fibrils accumulate, tissues and organs may fail to function properly. Evidence of amyloid deposition in duodenal biopsies and bone marrow (BM) aspirates is uncommon and often overlooked. Here, we present a patient diagnosed with primary systemic amyloidosis who complained of pain in the abdomen, vomiting, loose stools, and generalized weakness. Histological examination of an endoscopic duodenal biopsy revealed amyloid deposits. BM aspiration cytology revealed amyloid deposition with BM plasmacytosis. She was eventually diagnosed with plasma cell dyscrasia based on a series of biochemical tests. To the best of our knowledge, reports of simultaneous amyloid deposition in the duodenum and BM aspirate smears are very rare and unpublished. This case serves to highlight the significance of careful microscopic histo-cytology and the utility of special stains for prompt diagnosis and treatment outcomes in a disease of poor prognosis.
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一例原发性系统性淀粉样变性伴淀粉样蛋白沉积于十二指肠和骨髓吸出物:罕见发现
淀粉样变性是一组以细胞外异常蛋白质物质(淀粉样蛋白)沉积在各种器官为特征的疾病。随着淀粉样纤维的积累,组织和器官可能无法正常工作。十二指肠活检和骨髓(BM)抽吸中淀粉样蛋白沉积的证据并不常见,而且经常被忽视。在这里,我们介绍了一名被诊断为原发性系统性淀粉样变性的患者,他抱怨腹部疼痛、呕吐、便便疏松和全身无力。内镜十二指肠活检的组织学检查显示淀粉样蛋白沉积。骨髓抽吸细胞学检查显示淀粉样蛋白沉积伴骨髓浆细胞增多。根据一系列生化测试,她最终被诊断为浆细胞发育异常。据我们所知,十二指肠和骨髓抽吸涂片中同时存在淀粉样蛋白沉积的报道非常罕见且未发表。该病例强调了仔细的显微镜组织细胞学检查的重要性,以及特殊染色对预后不良疾病的及时诊断和治疗结果的实用性。
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审稿时长
17 weeks
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