Haematologica, Vol 92, Issue 6, e74-e76 doi:10.3324/haematol.11475
Copyright © 2007 by Ferrata Storti Foundation
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Online Only Articles

Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship

Niels P Riksen, MD, PhD1,2,, Brenda M Luken, PhD3, Ina S Klasen, PhD4, Jan Voorberg, PhD3, Niels Crama, MD5, Marcel van Deuren, MD, PhD1

1 Department of Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
2 Department of Pharmacology-Toxicology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
4 Department of Blood Transfusion and Transplantation Immunology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
5 Department of and Ophthalmology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
3 Department of Plasma Proteins, Sanquin Research and Joint Landsteiner Laboratory of Sanquin and Academic Medical Centre, University of Amsterdam, The Netherlands

Correspondence: Niels P Riksen, MD, PhD, Department of Pharmacology-Toxicology 149 Radboud University Nijmegen Medical Centre PO Box 9101 6500 HB NijmegenThe Netherlands, Phone: +31-24-3618819; Fax:+31-24-3614214, Email: N.Riksen{at}aig.umcn.nl

We present a patient with a history of benign monoclonal gammopathy, who developed thrombotic thrombocytopenic purpura (TTP), initially presenting as bilateral serous retinal detachment. Plasma of the patient contained high titers of anti-ADAMTS13 antibodies that were directed towards the disintegrin/TSR1/cysteine-rich/spacer and CUB1-2 domains. ADAMTS13 activity was undetectable. Total IgG purified from plasma of the patient partially inhibited ADAMTS13 activity. In contrast, the isolated M-protein did neither bind to, nor inhibit activity of ADAMTS13. We conclude that in this patient the monoclonal gammopathy and TTP co-existed as distinct pathological entities.

Key words: ADAMTS13, TTP, MGUS, antibodies, CUB domain, retinal detachment.