Suppr超能文献

原发性淀粉样变性——1例浆细胞计数正常的病例

Primary Amyloidosis - In a Case with Normal Plasma Cell Counts.

作者信息

Trehan Pooja, Gouri Mangala, Wilfred Clement, Rashmi K, Mysorekar Vijaya

机构信息

Postgraduate Student, Department of Pathology, MS Ramaiah Medical College, Bengaluru, Karnataka, India.

Professor, Department of Pathology, MS Ramaiah Medical College, Bengaluru, Karnataka, India.

出版信息

J Clin Diagn Res. 2017 Jul;11(7):ED21-ED23. doi: 10.7860/JCDR/2017/26429.10241. Epub 2017 Jul 1.

Abstract

Amyloidosis is a group of disease that is characterized by the deposition of extracellular abnormal proteinaceous material (amyloid), in various organs. Amyloidosis involving the liver is common and the radiological findings are often nonspecific. We present the case of a 40-year-old female who presented with abdominal pain. Ultrasound abdomen was reported as massive hepatomegaly with diffuse liver parenchymal disease. Bone marrow aspiration showed normomegaloblastic erythroid hyperplasia and plasma cells were within normal limits (5%). Also, amorphous, eosinophilic fragmented to smudgy material within the interstitium of cell trails was seen. Bone marrow biopsy and liver biopsy also showed similar kind of homogenous eosinophilic material. Both liver biopsy and bone marrow biopsy were subjected to special stains which confirmed the presence of amyloid. The patient did not have clinical or laboratory findings suggestive of any other organ involvement. Thus, we conclude that clinical and imaging presentations of amyloidosis are often nonspecific, hence biopsy is always required to confirm the diagnosis. Amyloid deposits on bone marrow aspiration are a rare occurrence and are often missed. It is an unusual sighting with very few studies mentioning its occurrence.

摘要

淀粉样变性是一组以细胞外异常蛋白质物质(淀粉样蛋白)在各种器官中沉积为特征的疾病。累及肝脏的淀粉样变性很常见,其影像学表现通常不具有特异性。我们报告一例40岁女性患者,该患者因腹痛就诊。腹部超声报告显示肝脏肿大且伴有弥漫性肝实质疾病。骨髓穿刺显示正成红细胞性红细胞增生,浆细胞在正常范围内(5%)。此外,在细胞条索间质内可见无定形、嗜酸性、破碎成模糊状的物质。骨髓活检和肝活检也显示出类似的均匀嗜酸性物质。肝活检和骨髓活检均进行了特殊染色,证实存在淀粉样蛋白。该患者没有提示任何其他器官受累的临床或实验室检查结果。因此,我们得出结论,淀粉样变性的临床和影像学表现通常不具有特异性,因此总是需要活检来确诊。骨髓穿刺时发现淀粉样沉积物的情况罕见,常常被漏诊。这是一种不常见的现象,很少有研究提及它的发生。

相似文献

1
Primary Amyloidosis - In a Case with Normal Plasma Cell Counts.原发性淀粉样变性——1例浆细胞计数正常的病例
J Clin Diagn Res. 2017 Jul;11(7):ED21-ED23. doi: 10.7860/JCDR/2017/26429.10241. Epub 2017 Jul 1.
2
Massive Hepatomegaly Secondary to Amyloidosis with Normal Liver Chemistries.淀粉样变性继发巨肝,肝功能正常
Case Rep Gastroenterol. 2020 May 13;14(2):271-278. doi: 10.1159/000507612. eCollection 2020 May-Aug.
4
Amyloidosis: an unusual cause of portal hypertension.淀粉样变性:门静脉高压的一种罕见病因。
Autops Case Rep. 2016 Jun 30;6(2):9-18. doi: 10.4322/acr.2016.035. eCollection 2016 Apr-Jun.

本文引用的文献

3
Extensive amyloid deposit in bone marrow aspirate.骨髓穿刺物中存在广泛的淀粉样沉积物。
Indian J Hematol Blood Transfus. 2013 Sep;29(3):187-8. doi: 10.1007/s12288-012-0161-z. Epub 2012 Jun 1.
7
Hepatic amyloidosis--two cases report.肝淀粉样变性——两例报告。
J Korean Med Sci. 1988 Dec;3(4):151-5. doi: 10.3346/jkms.1988.3.4.151.

文献检索

告别复杂PubMed语法,用中文像聊天一样搜索,搜遍4000万医学文献。AI智能推荐,让科研检索更轻松。

立即免费搜索

文件翻译

保留排版,准确专业,支持PDF/Word/PPT等文件格式,支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述,25分钟生成高质量综述,智能提取关键信息,辅助科研写作。

立即免费体验