Faculty Collaboration Database

Acquired factor X deficiency in light-chain (AL) amyloidosis is rare and associated with advanced disease. Hematol Oncol Stem Cell Ther 2019 Mar;12(1):10-14

Date

03/19/2019

Pubmed ID

30879471

DOI

10.1016/j.hemonc.2018.05.002

Scopus ID

2-s2.0-85052754317 (requires institutional sign-in at Scopus site)   20 Citations

Abstract

INTRODUCTION: Systemic light chain (AL) amyloidosis can lead to an acquired coagulopathy secondary to acquired factor X (aFX) deficiency. However, it is not very clear who develops aFX deficiency in AL amyloidosis.

METHODS: We therefore undertook this single centre, retrospective study to better characterize AL amyloidosis-associated aFX deficiency.

RESULTS: Out of 121 AL patients who had FX testing at the time of their first evaluation at our institution, including 17 patients on warfarin at the time of testing, 10 out of 104 patients (9.6%) with systemic AL amyloidosis were found to have FX levels below 50%. Acquired FX deficiency was associated with advanced stage of AL amyloidosis and elevated cardiac biomarkers. Lower FX activity, advanced stage, and cardiac involvement by disease were associated with higher hazard of death on univariate analysis. On multivariate analysis, stage of AL amyloidosis was the only significant predictor of survival. Median survival time of patients with FX deficiency was 9.3 months compared to 118.4 months in those without.

CONCLUSIONS: We conclude that while aFX deficiency is rare in systemic AL amyloidosis, it is a marker of advanced disease.

Author List

Patel G, Hari P, Szabo A, Rein L, Kreuziger LB, Chhabra S, Dhakal B, D'Souza A

Authors

Lisa M. Baumann Kreuziger MD Associate Professor in the Medicine department at Medical College of Wisconsin
Anita D'Souza MD Associate Professor in the Medicine department at Medical College of Wisconsin
Binod Dhakal MD Associate Professor in the Medicine department at Medical College of Wisconsin
Parameswaran Hari MD Adjunct Professor in the Medicine department at Medical College of Wisconsin
Lisa E. Rein Biostatistician III in the Institute for Health and Equity department at Medical College of Wisconsin
Aniko Szabo PhD Professor in the Institute for Health and Equity department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Adult
Aged
Aged, 80 and over
Disease-Free Survival
Factor X
Factor X Deficiency
Female
Humans
Immunoglobulin Light-chain Amyloidosis
Male
Middle Aged
Survival Rate