Activity of von Willebrand factor (VWF)-cleaving protease (ADAMTS13) in 68 patients with thrombocytopenia.

Multimer analysis of VWF substrate digested by diluted (1:20) plasma samples from patients. On each gel (A-H), a calibration curve using dilutions of pooled plasma from healthy donors (NHP; 1:20 dilution corresponding to 100%) is included. Seventeen patients with severe sepsis (gels A,B), 16 with heparin-induced thrombocytopenia (gels C,D), and 35 with thrombocytopenia due to various causes (gels E-H) were analyzed. Samples of digested VWF were applied on top of the gel.

OMF, osteomyelofibrosis; MDS, myelodysplastic syndrome; ITP, idiopathic thrombocytopenic purpura; AL, acute leukemia; SAA, severe aplastic anemia; Misc, miscellaneous causes.

Reprinted with permission from Bianchi V, et al. Von Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood. 2002;100:710–713.