Diffuse neurodeficits in intravascular lymphomatosis with ADAMTS13 inhibitor.
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.
Kawahara
M
M
Department of Neurology, Nara Medical University, Shijyo-cho 840, Kashihara city, Nara 634-8521, Japan. kawamm@naramed-u.ac.jp
Kanno
M
M
Matsumoto
M
M
Nakamura
S
S
Fujimura
Y
Y
Ueno
S
S
D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000097:blood; D001921:Brain / Q000473:pathology; D006801:Humans; D008168:Lung / Q000098:blood supply; D016393:Lymphoma, B-Cell / Q000150:complications / Q000175:diagnosis* / Q000276:immunology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008666:Metalloendopeptidases / Q000276:immunology*; D008875:Middle Aged; D009422:Nervous System Diseases / Q000175:diagnosis* / Q000209:etiology; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology; D019043:Vascular Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000276:immunology*; D014699:Venules / Q000473:pathology
D001323:Autoantibodies; D051722:ADAM Proteins; D008666:Metalloendopeptidases; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human