Prognostic factors in light chain amyloidosis

被引：4
作者：
Javorniczky Nora Rebeka [1 ]
Bodo Imre [2 ]
Masszi Tamas [3 ]
Mikala Gabor [3 ]
机构：
[1] Semmelweis Egyet, Altalanos Orvostud Kar, Budapest, Hungary
[2] Emory Univ, Sch Med, Atlanta, GA USA
[3] Egyesitett Szent Istvan & Szent Laszlo Korhaz, Budapest, Hungary
来源：
ORVOSI HETILAP | 2015年 / 156卷 / 39期
关键词：
amyloidosis; light chain; autologous stem cell transplantation; bortezomib; STEM-CELL TRANSPLANTATION; PRIMARY SYSTEMIC AMYLOIDOSIS; SUBCUTANEOUS FAT ASPIRATION; HIGH-DOSE MELPHALAN; AL AMYLOIDOSIS; MONOCLONAL GAMMOPATHIES; BORTEZOMIB; DEXAMETHASONE; DIAGNOSIS; INVOLVEMENT;
D O I：
10.1556/650.2015.30241
中图分类号：
R5 [内科学];
学科分类号：
1002 ; 100201 ;
摘要：
Introduction: Light chain amyloidosis is characterized by extracellular deposition of a fibrillar material derived from immunglobulin light chain fragments. Aim: The aim of the authors was to assess survival depending on cardiac involvement, therapy, and presence of myeloma. Method: The authors studied a retrospective cohort of 29 patients with light chain amyloidosis (13 kappa, 16 lambda) treated in their institution between 2005 and 2014. Results: Twenty-one patients had primary amyloidosis, while 8 had coexisting multiple myeloma. One, two and three or more organs were involved in 4, 8, and 17 patients, respectively. Cardiac involvement (22 cases) inversely correlated with survival. Fifteen (52%) patients received chemotherapy only, while 14 (48%) underwent autologous stem cell transplantation with a median survival of 87 and 11.4 months, respectively. Two patients had heart transplantation and survived 70 and 30 months. Median overall survival was 75.8 months. Conclusions: Cardiac transplantation followed by autologous stem cell transplantation is feasible in selected patients with light chain amyloidosis and heart failure.
引用
收藏
页码：1577 / 1584
页数：8
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