PMID: 2486537Sep 1, 1989Paper

Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

The American Journal of Medicine
Joel Moake, P D McPherson

Abstract

To analyze and review von Willebrand factor (vWF) multimeric patterns in patients with single-episode thrombotic thrombocytopenic purpura (TTP), intermittent TTP (episodes at infrequent, irregular intervals), chronic relapsing TTP (episodes at frequent, regular intervals), and the hemolytic-uremic syndrome (HUS). Platelet-poor plasma samples were obtained in EDTA, citrate, or citrate-hirudin-aprotinin-leupeptin from 36 patients with single-episode TTP, eight patients with intermittent TTP, four patients with chronic relapsing TTP, and 26 patients with HUS. The samples were separated by sodium dodecyl sulfate-agarose gel electrophoresis, overlaid with rabbit 125I-anti-human vWF IgG, and analyzed by autoradiography. Abnormalities of vWF multimers were found in platelet-poor plasma samples from 31 of 36 found in platelet-poor plasma samples from 31 of 36 patients (86%) at the onset of and during their single TTP episode. vWF multimers larger than those in normal plasma, and similar to vWF forms observed within normal human endothelial cells (unusually large vWF multimers), were demonstrated in 31% of the patients; 19% had either unusually large vWF multimers or a relative decrease in the largest plasma vWF forms in different seria...Continue Reading

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