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Prognostic significance of interventricular septal thickness in patients with AL amyloidosis

 Hyunsoo Cho  ;  Soo-Jeong Kim  ;  Chi Young Shim  ;  Geu-Ru Hong  ;  Jong-Won Ha  ;  Yu Ri Kim  ;  Woo Ick Yang  ;  Haerim Chung  ;  Ji Eun Jang  ;  June-Won Cheong  ;  Yoo Hong Min  ;  Jin Seok Kim 
 Leukemia Research, Vol.60 : 36-43, 2017 
Journal Title
 Leukemia Research 
Issue Date
Adult ; Aged ; Amyloidosis/complications ; Amyloidosis/diagnosis ; Amyloidosis/pathology ; Female ; Heart Neoplasms/pathology ; Heart Septum/pathology ; Heart Ventricles/pathology ; Humans ; Immunoglobulin Light Chains ; Immunoglobulin Light-chain Amyloidosis ; Male ; Middle Aged ; Prognosis ; Retrospective Studies
AL amyloidosis ; Cardiac involvement ; Interventricular septal thickness ; Prognostic factor
The major prognostic determinant of immunoglobulin light chain (AL) amyloidosis is cardiac involvement. However, the role of interventricular septal thickness (IVST), which reflects the extent of cardiac involvement, remains unclear. Therefore, we analyzed 77 patients with newly diagnosed AL amyloidosis and evaluated the prognostic role of IVST. Fifty patients (64.9%) had cardiac involvement and 17 patients (22.1%) showed IVST >15mm. Among all patients, the revised Mayo Clinic Stage III-IV and IVST >15mm were independently associated with inferior overall survival (OS) in a multivariable analysis. IVST >15mm was also adversely prognostic for OS in a subgroup of advanced-stage (revised Mayo Clinic stage III-IV) patients in a multivariable analysis (P<0.001). Furthermore, advanced-stage patients with IVST >15mm did not show survival benefit from treatment with bortezomib-based regimens and/or autologous stem-cell transplantation (ASCT). Our study demonstrated that IVST >15mm is adversely prognostic independent of the revised Mayo Clinic staging system in patients with AL amyloidosis. In addition, the degree of IVST might be used as a useful prognostic indicator that can guide the management of patients with AL amyloidosis especially at an advanced stage.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
김수정(Kim, Soo Jeong) ORCID logo https://orcid.org/0000-0001-8859-3573
김유리(Kim, Yu Ri) ORCID logo https://orcid.org/0000-0001-5505-0142
김진석(Kim, Jin Seok) ORCID logo https://orcid.org/0000-0001-8986-8436
민유홍(Min, Yoo Hong) ORCID logo https://orcid.org/0000-0001-8542-9583
심지영(Shim, Chi Young) ORCID logo https://orcid.org/0000-0002-6136-0136
양우익(Yang, Woo Ick) ORCID logo https://orcid.org/0000-0002-6084-5019
장지은(Jang, Ji Eun) ORCID logo https://orcid.org/0000-0001-8832-1412
정준원(Cheong, June-Won) ORCID logo https://orcid.org/0000-0002-1744-0921
정해림(Chung, Hae Rim) ORCID logo https://orcid.org/0000-0002-7926-9285
하종원(Ha, Jong Won) ORCID logo https://orcid.org/0000-0002-8260-2958
홍그루(Hong, Geu Ru) ORCID logo https://orcid.org/0000-0003-4981-3304
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