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伴有因子X缺乏的严重肝功能不全的免疫球蛋白轻链淀粉样变性

Immunoglobulin Light Chain Amyloidosis with Severe Liver Dysfunction Accompanied by Factor X Deficiency.

作者信息

Guo Yong-Mei, Takahashi Nagi, Miyabe Ken, Yoshida Makoto, Abe Fumito, Yamashita Takaya, Nara Miho, Yoshioka Tomoko, Ohashi Kenichi, Goto Akiteru, Takahashi Naoto

机构信息

Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan.

Department of Cellular and Organ Pathology, Akita University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2019 Oct 15;58(20):3039-3043. doi: 10.2169/internalmedicine.2864-19. Epub 2019 Jun 27.

DOI:10.2169/internalmedicine.2864-19
PMID:31243220
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6859402/
Abstract

Severe hepatic failure is rarely a cause of death in patients with immunoglobulin light chain (AL) amyloidosis. We herein report a case of AL amyloidosis involving a bleeding tendency due to factor X deficiency and marked hepatic involvement of amyloidosis. The patient died due to severe liver dysfunction two weeks after admission. The diagnosis was confirmed histologically by AL-λ amyloidosis, with the liver and spleen as the main lesions, on an autopsy. As treatment-related toxicity is strong in advanced cases, appropriate treatments are required to improve the prognosis of AL amyloidosis with severe liver dysfunction.

摘要

严重肝衰竭在免疫球蛋白轻链（AL）淀粉样变性患者中很少是死亡原因。我们在此报告一例AL淀粉样变性病例，该患者因X因子缺乏而有出血倾向，且淀粉样变性有明显肝脏受累。患者入院两周后因严重肝功能障碍死亡。尸检组织学检查证实诊断为AL-λ淀粉样变性，以肝脏和脾脏为主要病变部位。由于晚期病例中与治疗相关的毒性很强，因此需要采取适当治疗以改善伴有严重肝功能障碍的AL淀粉样变性患者的预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dd6/6859402/5e8d524f1e10/1349-7235-58-3039-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dd6/6859402/c10d65ab01e5/1349-7235-58-3039-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dd6/6859402/5e8d524f1e10/1349-7235-58-3039-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dd6/6859402/c10d65ab01e5/1349-7235-58-3039-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dd6/6859402/5e8d524f1e10/1349-7235-58-3039-g002.jpg

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引用本文的文献

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本文引用的文献

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Direct Factor X sequestration by systemic amyloid light-chain amyloidosis.系统性轻链型淀粉样变性对凝血因子X的直接隔离作用
Clin Case Rep. 2018 Jan 31;6(3):513-515. doi: 10.1002/ccr3.1398. eCollection 2018 Mar.
2
Nationwide Survey of 741 Patients with Systemic Amyloid Light-chain Amyloidosis in Japan.日本741例系统性轻链型淀粉样变患者的全国性调查。
Intern Med. 2018 Jan 15;57(2):181-187. doi: 10.2169/internalmedicine.9206-17. Epub 2017 Nov 1.
3
Liver transplantation followed by autologous stem cell transplantation for acute liver failure caused by AL amyloidosis. Case report and review of the literature.
肝移植后自体干细胞移植治疗AL淀粉样变性所致急性肝衰竭。病例报告及文献复习。
Ann Hepatol. 2016 Jul-Aug;15(4):592-7.
4
Immunoglobulin light chain amyloidosis: 2013 update on diagnosis, prognosis, and treatment.免疫球蛋白轻链淀粉样变性：2013 年诊断、预后和治疗更新。
Am J Hematol. 2013 May;88(5):416-25. doi: 10.1002/ajh.23400.
5
What do I need to know about immunoglobulin light chain (AL) amyloidosis?我需要了解免疫球蛋白轻链（AL）淀粉样变性的哪些知识？
Blood Rev. 2012 Jul;26(4):137-54. doi: 10.1016/j.blre.2012.03.001. Epub 2012 Apr 25.
6
Outcome of AL amyloidosis after high-dose melphalan and autologous stem cell transplantation: long-term results in a series of 421 patients.大剂量美法仑和自体干细胞移植后 AL 淀粉样变性的结果：一系列 421 例患者的长期结果。
Blood. 2011 Oct 20;118(16):4346-52. doi: 10.1182/blood-2011-01-330738. Epub 2011 Aug 9.
7
Hepatic response after high-dose melphalan and stem cell transplantation in patients with AL amyloidosis associated liver disease.伴肝脏疾病的AL淀粉样变性患者接受大剂量美法仑和干细胞移植后的肝脏反应
Haematologica. 2009 Jul;94(7):1029-32. doi: 10.3324/haematol.2008.001925. Epub 2009 May 19.
8
Successful treatment of systemic amyloidosis with hepatic involvement and factor X deficiency by high dose melphalan chemotherapy and autologous stem cell reinfusion.
Eur J Haematol. 2004 Mar;72(3):181-5. doi: 10.1046/j.0902-4441.2003.00200.x.
9
Primary (AL) hepatic amyloidosis: clinical features and natural history in 98 patients.原发性（AL）肝淀粉样变性：98例患者的临床特征及自然病史
Medicine (Baltimore). 2003 Sep;82(5):291-8. doi: 10.1097/01.md.0000091183.93122.c7.
10
Bleeding symptoms and coagulation abnormalities in 337 patients with AL-amyloidosis.337例AL型淀粉样变性患者的出血症状及凝血异常
Br J Haematol. 2000 Aug;110(2):454-60. doi: 10.1046/j.1365-2141.2000.02183.x.