Clinical and prognostic significance of serum levels of von Willebrand factor and ADAMTS-13 antigens in AL amyloidosis

Author:

Kastritis Efstathios1,Papassotiriou Ioannis2,Terpos Evangelos1,Roussou Maria1,Gavriatopoulou Maria1,Komitopoulou Anna3,Skevaki Chrysanthi2,Eleutherakis-Papaiakovou Evangelos1,Pamboucas Constantinos1,Psimenou Erasmia1,Manios Efstathios1,Giannouli Stavroula4,Politou Marianna5,Gakiopoulou Harikleia6,Papadopoulou Elektra1,Stamatelopoulos Kimon1,Tasidou Anna7,Dimopoulos Meletios A.1

Affiliation:

1. Department of Clinical Therapeutics, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;

2. Department of Clinical Biochemistry, and

3. Hemostasis Unit, Hemophilia Centre, Aghia Sophia Children's Hospital, Athens, Greece;

4. Second Department of Internal Medicine, National and Kapodistrian University of Athens School of Medicine, Athens, Greece;

5. Hematology Laboratory, Blood Bank, Aretaieion Hospital, Athens, Greece;

6. First Department of Pathology, National and Kapodistrian University of Athens School of Medicine, Athens, Greece; and

7. Department of Haemopathology, Evangelismos Hospital, Athens, Greece

Abstract

Key Points Serum levels of VWF antigen are elevated in AL amyloidosis, reflecting endothelial dysfunction. High VWF levels predict for poor outcome in patients with cardiac involvement and discriminate high-risk patients even within stage IIIB.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

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