N<scp>EW</scp>A<scp>SPECTS</scp><scp>IN</scp><scp>THE</scp>P<scp>ATHOGENESIS</scp><scp>AND</scp>T<scp>REATMENT</scp><scp>OF</scp>T<scp>HROMBOTIC</scp>T<scp>HROMBOCYTOPENIC</scp>P<scp>URPURA</scp><scp>AND</scp>H<scp>EMOLYTIC</scp>U<scp>REMIC</scp>S<scp>YNDROME</scp>

Raife, Thomas J.; Montgomery, Robert K.

doi:10.1046/j.1468-0734.2001.00044.x

Cited by 10 publications

(6 citation statements)

References 183 publications

(217 reference statements)

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“…Equally important, the translocation of PLTs after a few seconds suggested the existence of a regulatory process that released the PLTs. These observations led to speculation that the observed PLT adhesion to endothelium could be the basis of a model of thrombosis in TTP if combined with a pathological dysfunction of the release mechanism 34 …”

Section: Evidence For a New Model Of Ttp Microvascular Thrombosismentioning

confidence: 99%

“…The mouse model and capillary flow chamber studies led to new ideas about the VWF regulatory function of ADAMTS13 and to a revised model of TTP microvascular thrombosis 34,37‐39 . These studies offered direct evidence that PLTs bind to stimulated endothelial cells via emerging VWF and that the process is regulated by ADAMTS13, which releases PLTs as it cleaves the VWF.…”

Section: Microvascular Thrombosis At the Endothelial Surfacementioning

confidence: 99%

See 1 more Smart Citation

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

Raife

Friedman²,

Dwyre³

2005

Transfusion

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New developments in the understanding of thrombotic thrombocytopenic purpura (TTP) provide opportunities for improved patient care. A widely held historical model of TTP microvascular thrombosis implicated circulating ultra large von Willebrand factor (ULVWF) in causing spontaneous platelet (PLT) aggregation. From this pathogenic model, concerns about ULVWF in fresh-frozen plasma (FFP) used to treat patients led to widespread use of cryopoor plasma (CPP) as an alternative. There is scant evidence, however, that circulating ULVWF contributes to microvascular thrombosis in TTP. New evidence suggests that the formation of PLT aggregates in TTP may be mediated by VWF in the process of being released from endothelium. Moreover, clinical studies do not demonstrate superior efficacy of CPP compared to FFP in the treatment of TTP. Because CPP may have reduced concentrations of factors important in the treatment of TTP, including ADAMTS13 metalloprotease, a reappraisal of the use of CPP in the treatment of TTP is warranted.

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Section: Evidence For a New Model Of Ttp Microvascular Thrombosismentioning

confidence: 99%

Section: Microvascular Thrombosis At the Endothelial Surfacementioning

confidence: 99%

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

Raife

Friedman²,

Dwyre³

2005

Transfusion

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“…Another possibility is abnormally functioning hemostatic mechanisms. These may include genetic defects in platelet function, coagulation, and fibrinolysis (Raife et al, 2001).…”

Section: Thrombotic Microangiopathymentioning

confidence: 99%

Genetic abnormalities of the endothelium

Butany

Verma

Leask

et al. 2002

Microscopy Res & Technique

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Endothelial cell dysfunction plays an important role in the development and progression of cardiovascular and other disease. The purpose of this review is to discuss some of the genetic diseases known to adversely affect endothelial function. Although the list is exhaustive, we focus our discussion on primary pulmonary hypertension, diabetes mellitus, Alzheimer's disease, Crohn's disease, Von-Hippel-Lindau disease, familial Mediterranean fever, thrombotic microangiopathy, and key vascular malformations. Endothelial dysfunction results from a complex interplay between genetic and environmental factors. This review highlights some of the growing body of evidence implicating endothelial dysfunction as an important mediator of diverse diseases.

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“…Currently there are no specific protective measures or therapy against STEC infection other than supportive therapy; the utility of antibiotics or antidiarrhetics is uncertain, and they may even be contraindicated (117,138). Several excellent publications provide a comprehensive review of the current knowledge on these pathogens and the sequelae of STEC-induced HUS (2,95,102,104,119).…”

Section: Introductionmentioning

confidence: 99%

Antibody Therapy in the Management of Shiga Toxin-Induced Hemolytic Uremic Syndrome

et al. 2004

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Hemolytic uremic syndrome (HUS) is a disease that can lead to acute renal failure and often to other serious sequelae, including death. The majority of cases are attributed to infections with Escherichia coli, serotype O157:H7 strains in particular, which cause bloody diarrhea and liberate one or two toxins known as Shiga toxins 1 and 2. These toxins are thought to directly be responsible for the manifestations of HUS. Currently, supportive nonspecific treatment is the only available option for the management of individuals presenting with HUS. The benefit of antimicrobial therapy remains uncertain because of several reports which claim that such intervention can in fact exacerbate the syndrome. There have been only a few specific therapies directed against neutralizing the activities of these toxins, but none so far has been shown to be effective. This article reviews the literature on the mechanism of action of these toxins and the clinical manifestations and current management and treatment of HUS. The major focus of the article, however, is the development and rationale for using neutralizing human antibodies to combat this toxin-induced disease. Several groups are currently pursuing this approach with either humanized, chimeric, or human antitoxin antibodies produced in transgenic mice. They are at different phases of development, ranging from preclinical evaluation to human clinical trials. The information available from preclinical studies indicates that neutralizing specific antibodies directed against the A subunit of the toxin can be highly protective. Such antibodies, even when administered well after exposure to bacterial infection and onset of diarrhea, can prevent the occurrence of systemic complications

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NEWASPECTSINTHEPATHOGENESISANDTREATMENTOFTHROMBOTICTHROMBOCYTOPENICPURPURAANDHEMOLYTICUREMICSYNDROME

Cited by 10 publications

References 183 publications

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

The pathogenicity of von Willebrand factor in thrombotic thrombocytopenic purpura: reconsideration of treatment with cryopoor plasma

Genetic abnormalities of the endothelium

Antibody Therapy in the Management of Shiga Toxin-Induced Hemolytic Uremic Syndrome

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