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[Prognostic factors in light chain amyloidosis].

Authors :
Jávorniczky NR
Bodó I
Masszi T
Mikala G
Source :
Orvosi hetilap [Orv Hetil] 2015 Sep 27; Vol. 156 (39), pp. 1577-84.
Publication Year :
2015

Abstract

Introduction: Light chain amyloidosis is characterized by extracellular deposition of a fibrillar material derived from immunglobulin light chain fragments.<br />Aim: The aim of the authors was to assess survival depending on cardiac involvement, therapy, and presence of myeloma.<br />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.<br />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.<br />Conclusions: Cardiac transplantation followed by autologous stem cell transplantation is feasible in selected patients with light chain amyloidosis and heart failure.

Details

Language :
Hungarian
ISSN :
0030-6002
Volume :
156
Issue :
39
Database :
MEDLINE
Journal :
Orvosi hetilap
Publication Type :
Academic Journal
Accession number :
26550915
Full Text :
https://doi.org/10.1556/650.2015.30241