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

被引：2

作者：

Guo, Yong-mei ^{[1
]}

Takahashi, Nagi ^{[1
]}

Miyabe, Ken ^{[2
]}

Yoshida, Makoto ^{[2
]}

Abe, Fumito ^{[1
]}

Yamashita, Takaya ^{[1
]}

Nara, Miho ^{[1
]}

Yoshioka, Tomoko ^{[1
]}

Ohashi, Kenichi ^{[3
]}

Goto, Akiteru ^{[2
]}

Takahashi, Naoto ^{[1
]}

机构：

[1] Akita Univ, Grad Sch Med, Dept Hematol Nephrol & Rheumatol, Akita, Japan

[2] Akita Univ, Grad Sch Med, Dept Cellular & Organ Pathol, Akita, Japan

[3] Yokohama City Univ, Grad Sch Med, Dept Pathol, Yokohama, Kanagawa, Japan

来源：

INTERNAL MEDICINE | 2019年 / 58卷 / 20期

基金：

日本学术振兴会;

关键词：

AL amyloidosis; hepatic amyloidosis; liver dysfunction; bleeding tendency; factor X; STEM-CELL TRANSPLANTATION; HIGH-DOSE MELPHALAN; HEPATIC AMYLOIDOSIS; AL AMYLOIDOSIS; NATURAL-HISTORY;

D O I：

10.2169/internalmedicine.2864-19

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

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-lambda 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.

引用

页码：3039 / 3043

页数：5

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