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NEUROLOGY 2004;63:1731-1733
© 2004 American Academy of Neurology
Brief Communications

Diffuse neurodeficits in intravascular lymphomatosis with ADAMTS13 inhibitor

M. Kawahara, MD, M. Kanno, MD, PhD, M. Matsumoto, MD, PhD, S. Nakamura, MD, PhD, Y. Fujimura, MD, PhD and S. Ueno, MD, PhD

From the Departments of Neurology (Drs. Kawahara and Ueno), General Medicine (Drs. Kanno and Nakamura), and Blood Transfusion Medicine (Drs. Matsumoto and Fujimura), Nara Medical University, Kashihara, Nara, Japan.

Address correspondence and reprint requests to Dr. Makoto Kawahara, Department of Neurology, Nara Medical University, Shijyo-cho 840, Kashihara city, Nara 634–8521, Japan; e-mail: kawamm{at}naramed-u.ac.jp

Inhibitory antibody to von Willebrand factor (vWF)-cleaving protease (ADAMTS13) was detected in a patient with intravascular lymphomatosis. The increased serum level of the antibody paralleled an increase in the expression of uncleaved vWF, which might cause microvascular thrombosis and platelet consumption. Malignant cell proliferations with superimposed thrombosis within the lumina throughout the entire vasculature account for diffuse neurodeficits observed in the patient.

Received February 13, 2004. Accepted in final form June 22, 2004.






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