Clinical features in the patients with hepatic amyloidosis

doi:10.3877/cma.j.issn.2095-7157.2020.03.005

Abstract

Abstract:

Objective

To summarize the clinical characteristics of patients with amyloidosis and guide the clinical diagnosis and treatment.

Methods

The clinical data of 12 patients with amyloidosis confirmed by pathological biopsy in Beijing You′an Hospital from January 2009 to December 2017 were retrospectively analyzed.

Results

There were 9 males and 3 females with an average age of (56±8) years (range, 39-70 years). Clinical manifestations: fatigue in 5 cases, poor appetite in 4 cases, abdominal distension in 6 cases, abdominal pain in 6 cases, ascites in 6 cases, hepatomegaly in 6 cases, splenomegaly in 4 cases. Laboratory examination: 6 cases of abnormal liver function, 4 cases of abnormal transaminase, 4 cases of elevated bilirubin, 10 cases of elevated gamma glutamyltranspeptidase, 10 cases of elevated alkaline phosphatase, 8 cases of hypoalbuminemia, 8 cases of proteinuria, 2 cases of hematuria, 6 cases of anemia.

Conclusion

Amyloidosis is more common in men with various clinical manifestations and liver and kidney damage, which is usually diagnosed by pathological biopsy.

Key words: Amyloidosis, Liver injury, Renal injury, Pathological biopsy

Wen Gao, Rui Zhong, Man Zhao, Dongmei Wang, Weiwei Kang, Lili Feng, Hongbo Ma, Lieqing Ma. Clinical features in the patients with hepatic amyloidosis[J]. Chinese Journal of Gastrointestinal Endoscopy(Electronic Edition), 2020, 07(03): 126-129.

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Figures/Tables 3

References 13

[1]	余思邈，王亚超，朱云，等.12例肝淀粉样变性患者临床特征分析[J]．临床肝胆病杂志，2018，34(4)：836-839.
[2]	范春蕾，王征，李磊，等．以巨肝为主要表现的原发性系统性淀粉样变性1例报告[J]．临床肝胆病杂志，2016，32(9)：1791-1793.
[3]	Qiu Y, Zhang CL, Shen KN，et al.Clinical presentation and prognosis of light-chain amyloidosis patients with unmeasurable free light-chain levels[J]．Ann Hematol，2018，97(12)：2465-2470.
[4]	中国系统性淀粉样变性协作组．系统性轻链型淀粉样变性诊断和治疗指南[J]. 中华医学杂志，2016，96(44)：3540-3548.
[5]	Yao Y, Wang SX, Zhang YK，et al.A clinicopathological analysis in a large cohort of Chinese patients with renal amyloid light-chain amyloidosis[J]．Nephrol Dial Transplant，2013，28(3)：689-697.
[6]	Ichimata S, Kobayashi M, Shimojo H，et al.Usefulness of gastroduodenal biopsy in the differential diagnosis of systemic AH amyloidosis from systemic AL amyloidosis[J]．Histopathology，2018，73(2)：230-239.
[7]	姚健，张国艳，刘玉兰．肝脏淀粉样变性[J]．胃肠病学及肝病学杂志，2004，13(2)：191-192.
[8]	Kyle RA, Gertz MA.Primary systemic amyloidosis: clinical and laboratory features in 474 cases[J]．Semin Hematol，1995，32(1)：45-59.
[9]	Correction：utility of abdominal skin plus subcutaneous fat and rectal mucosal biopsy in the diagnosis of AL amyloidosis with renal involvement[J]．PLoS ONE，2017，12(11)：e0187909.
[10]	Miyazaki K, Kawai S, Suzuki K．Abdominal subcutaneous fat pad aspiration and bone marrow examination for the diagnosis of AL amyloidosis：the reliability of immunohistochemistry[J]．Int J Hematol，2015，102(3)：289-295.
[11]	Picken MM.Modern approaches to the treatment of amyloidosis：the critical importance of early detection in surgical pathology[J]．Adv Anat Pathol，2013，20(6)：424-439.
[12]	Lobato L, Rocha A．Transthyretin amyloidosis and the kidney[J]．Clin J Am Soc Nephrol，2012，7(8)：1337-1346.
[13]	Kumar S, Dispenzieri A, Katzmann JA，et al.Serum immunoglobulin free light-chain measurement in primary amyloidosis：prognostic value and correlations with clinical features[J]．Blood，2010，116(24): 5126-5129.

项目			构成比/发生率[n(%)]	均数±标准差	范围
血红蛋白(g/L)			?	126±31.3	78～177
?		≤60	0(0)	?	?
?		≤90	1(8.33)	?	?
?		≤120	5(41.6)	?	?
白细胞(×10⁹/L)			?	7.32±1.57	4.5～9.23
血小板(×10⁹/L)			?	246±151	36～477
?		≤400	10(83.3)	?	?
?		>400	2(16.7)	?	?
血肌酐(μmol/L)			?	126.7±72.3	48～274.3
?		≥97	6(50)	?	?
AST(u/L)			?	33±17.7	12～66.8
?		≥40	4(33.3)	?	?
总胆红素(μmol/L)			?	77.3±156	6.1～538
直接胆红素(μmol/L)			?	33.6±67	3～224
γ谷氨酰转肽酶(U/L)			?	757.8±966	38～3128
碱性磷酸酶(U/L)			?	413.3±241.9	84～847.6
C反应蛋白(mg/L)			?	1.3±1.6	0.3～2.7
凝血酶原活动度(%)			5(41.67)	?	?
?	<80		5(41.67)	?	?
尿蛋白阳性(g/L)			3(25)	1.46±10.3	0.08～3.54
?	>1		3(25)	1.46±10.3	0.08～3.54

项目			构成比/发生率[n(%)]	均数±标准差	范围
血红蛋白(g/L)			?	126±31.3	78～177
?		≤60	0(0)	?	?
?		≤90	1(8.33)	?	?
?		≤120	5(41.6)	?	?
白细胞(×10⁹/L)			?	7.32±1.57	4.5～9.23
血小板(×10⁹/L)			?	246±151	36～477
?		≤400	10(83.3)	?	?
?		>400	2(16.7)	?	?
血肌酐(μmol/L)			?	126.7±72.3	48～274.3
?		≥97	6(50)	?	?
AST(u/L)			?	33±17.7	12～66.8
?		≥40	4(33.3)	?	?
总胆红素(μmol/L)			?	77.3±156	6.1～538
直接胆红素(μmol/L)			?	33.6±67	3～224
γ谷氨酰转肽酶(U/L)			?	757.8±966	38～3128
碱性磷酸酶(U/L)			?	413.3±241.9	84～847.6
C反应蛋白(mg/L)			?	1.3±1.6	0.3～2.7
凝血酶原活动度(%)			5(41.67)	?	?
?	<80		5(41.67)	?	?
尿蛋白阳性(g/L)			3(25)	1.46±10.3	0.08～3.54
?	>1		3(25)	1.46±10.3	0.08～3.54

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