Shear stress-induced platelet aggregation in various types of von Willebrand disease.
We here examined the shear stress-induced platelet aggregation (SIPA) in various types of von Willebrand Disease (vWD), using a novel cone-and-plate viscometer recently developed by Ikeda et al. [1]. Under experimental conditions of high shear stress (90-108 dyne/cm2) known to induce von Willebrand factor (vWF)-dependent platelet aggregation in normal individuals, a variety of SIPA patterns, from a normal to total defect, were observed in 6 patients with type I vWD, while SIPA was totally absent in all 7 type IIA patients examined. One patient with Normandy type vWD exhibited a normal SIPA pattern under these experimental conditions. These findings, together with the vWF parameters of each patient, suggested that the presence of higher molecular weight multimers of plasma vWF, as well as an appropriate level of vWF antigen, is essential for SIPA to occur under high shear stress. Interestingly, vWF-dependent SIPA in 3 patients with type IIB vWD was found to be initiated by a low shear stress force, which did not induce such platelet aggregation in normal individuals. This might explain the intravessel platelet clumping that occurs, resulting in the intermittent thrombocytopenia observed in this type of the disease.
Nakagawa-Nishimura
Y
Y
Department of Pediatrics and Blood Transfusion, Nara Medical University, Japan.
Sugimoto
M
M
Tsuji
S
S
Kuwahara
M
M
Nishio
K
K
Takahashi
Y
Y
Fujimura
Y
Y
Murata
M
M
Handa
M
M
Ikeda
Y
Y
D000293:Adolescent; D000328:Adult; D000818:Animals; D000911:Antibodies, Monoclonal / Q000276:immunology; D002648:Child; D000939:Epitopes / Q000276:immunology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology; D008297:Male; D051379:Mice; D008875:Middle Aged; D008970:Molecular Weight; D010974:Platelet Aggregation; D010980:Platelet Membrane Glycoproteins / Q000378:metabolism; D011956:Receptors, Cell Surface / Q000378:metabolism; D013314:Stress, Mechanical; D014842:von Willebrand Diseases / Q000097:blood* / Q000145:classification; D014841:von Willebrand Factor / Q000737:chemistry / Q000276:immunology
D000911:Antibodies, Monoclonal; D000939:Epitopes; D007074:Immunoglobulin G; D010980:Platelet Membrane Glycoproteins; D011956:Receptors, Cell Surface; D014841:von Willebrand Factor; C028254:von Willebrand factor receptor