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. 2000 Jul;53(7):497–501. doi: 10.1136/jcp.53.7.497

Current understanding of the pathophysiology of thrombotic thrombocytopenic purpura

S Allford 1, S Machin 1
PMCID: PMC1731230  PMID: 10961171

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Figure 1 Peripheral blood film of patient with intermittent thrombotic thrombocytopenic purpura (TTP). Severe thrombocytopenia and red cell fragmentation are prominent features. Circulating normoblasts and neutrophilia may also be seen.

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Figure 2 Typical histological findings of acute thrombotic thrombocytopenic purpura (TTP) (cardiac muscle). There is florid thrombosis within the microvasculature. Immunohistochemistry shows these thrombi to be composed predominantly of von Willebrand factor (vWF) and platelets, with only small amounts of fibrin and fibrinogen.

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Figure 3 von Willebrand factor (vWF) multimeric analysis demonstrating unusually large forms of vWF (ULvWF) in patient plasma obtained during an episode of thrombotic thrombocytopenic purpura (TTP) (lane 1, marked with an arrow). By comparison, lane 2 shows the series of vWF multimeric bands present in normal pooled platelet poor plasma: ULvWF are not a feature.

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Figure 4 Proposed mechanism for proteolytic breakdown of von Willebrand factor (vWF). Ultra large forms of vWF (ULvWF) are reversibly converted in vitro to those forms normally found circulating in plasma by an activity in the cryosupernatant fraction of normal plasma with properties of a limited disulphide bond reductase. Under the conditions shown, a metalloproteinase further cleaves vWF forms into circulating 140 kDa and 176 kDa fragments, thus preventing reversible re-formation of ULvWF. This metalloproteinase activity has been reported to be absent in chronic relapsing thrombotic thrombocytopenic purpura (TTP), whereas it appears to be inhibited in single episode or intermittent adult TTP.

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

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