Predictors of survival in patients with systemic light-chain amyloidosis and cardiac involvement initially ineligible for stem cell transplantation and treated with oral melphalan and dexamethasone

Abstract High-dose melphalan (HDM) plus stem cell transplantation is an effective treatment for light-chain amyloidosis (AL), but is associated with high treatment-related mortality in patients with cardiac involvement. We studied 187 patients with cardiac involvement with AL who underwent HDM between 1996 and 2008. The median age was 57 years and the median time from diagnosis to HDM was 3.6 months. Half of the patients received reduced-dose melphalan (100-160 mg/m2). The median overall survival (OS) was 66 months, 54 months from diagnosis and HDM, respectively, and 91 patients (49%) were alive at the last follow-up 52 months (median) from HDM. Thirty patients (16%) died within 100 days of transplantation; only low serum albumin predicted early deaths. Overall, hematologic response (HR) and cardiac responses were seen in 66% and 41% of patients, respectively. The median OS for patients with and without HR was not reached and 22 months, respectively (P < .01); and for those with any decrease and no decrease in N-terminal-pro-brain natriuretic peptide was not reached and 26 months, respectively (P < .01). In multivariate analysis of baseline factors, only reduced-dose melphalan predicted shorter OS. HDM is feasible in patients with cardiac amyloidosis, and achievement of HR and organ response is associated with improved survival.

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Frequencies and Types of Arrhythmias in Patients With Systemic Light-Chain Amyloidosis With Cardiac Involvement Undergoing Stem Cell Transplantation on Telemetry Monitoring

The American Journal of Cardiology ◽

10.1016/j.amjcard.2009.05.040 ◽

2009 ◽

Vol 104 (7) ◽

pp. 990-994 ◽

Cited By ~ 31

Author(s):

Yuliya B. Goldsmith ◽

Jennifer Liu ◽

Joanne Chou ◽

James Hoffman ◽

Raymond L. Comenzo ◽

...

Keyword(s):

Stem Cell ◽

Stem Cell Transplantation ◽

Cell Transplantation ◽

Light Chain ◽

Cardiac Involvement ◽

Light Chain Amyloidosis

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Combined orthotopic heart transplantation followed by autologous stem cell transplantation in a patient with light chain amyloidosis and isolated cardiac involvement

Cor et Vasa ◽

10.1016/j.crvasa.2017.10.004 ◽

2018 ◽

Vol 60 (5) ◽

pp. e518-e521 ◽

Cited By ~ 1

Author(s):

Vito Maurizio Parato ◽

Daniela Clemente ◽

Francesca Muscente ◽

Michele Scarano ◽

Ugolino Livi ◽

...

Keyword(s):

Stem Cell ◽

Stem Cell Transplantation ◽

Heart Transplantation ◽

Cell Transplantation ◽

Autologous Stem Cell Transplantation ◽

Light Chain ◽

Cardiac Involvement ◽

Orthotopic Heart Transplantation ◽

Light Chain Amyloidosis

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Prognosztikai tényezők könnyűlánc-amyloidosisban

Orvosi Hetilap ◽

10.1556/650.2015.30241 ◽

2015 ◽

Vol 156 (39) ◽

pp. 1577-1584

Author(s):

Nóra Rebeka Jávorniczky ◽

Imre Bodó ◽

Tamás Masszi ◽

Gábor Mikala

Keyword(s):

Stem Cell ◽

Stem Cell Transplantation ◽

Cell Transplantation ◽

Autologous Stem Cell Transplantation ◽

Light Chain ◽

Median Overall Survival ◽

Cardiac Involvement ◽

Primary Amyloidosis ◽

Light Chain Amyloidosis ◽

Fibrillar Material

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. Orv. Hetil., 2015, 156(39), 1577–1584.

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