Full evaluation of an acquired case of thrombotic thrombocytopenic purpura following the surgical resection of glioblastoma multiforme

Cataland, Spero R.; Jin, Ming; Smith, Edwin J.; Stanek, Michael; Wu, Haifeng

doi:10.1111/j.1538-7836.2006.02217.x

Cited by 9 publications

(6 citation statements)

References 46 publications

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“…Most of these case reports are described in the pre-ADAMTS13 era. Therefore, there is not much data on ADAMTS13 deficiency and postsurgical TTP except for recent ones where enzyme deficiency was identified in one case [28] but not the other [29]. Generally, there is no relapse of TTP once the postsurgical event is treated.…”

Section: Ttp Following Florid Inflammatory Conditionsmentioning

confidence: 86%

Atypical presentations of thrombotic thrombocytopenic purpura: A review

Sarode

2008

J of Clinical Apheresis

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Thrombotic thrombocytopenic purpura (TTP) is diagnosed by the presence of microangiopathic hemolytic anemia and thrombocytopenia in a patient who frequently presents with central nervous system involvement and, to a lesser extent, renal dysfunction. Recent understanding of the pathophysiology of TTP due to severe deficiency of von Willebrand factor cleaving protease, known as ADAMTS13, has improved diagnosis of TTP. Once the diagnosis is suspected, life-saving therapeutic plasma exchange therapy is initiated. Occasionally, an unusual clinical presentation makes TTP diagnosis difficult, thus resulting in a delay in the management of TTP. This review highlights a variety of atypical TTP presentations described in the literature. It is intended to bring unusual scenarios to the clinician's awareness, so that timely treatment can be delivered.

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Section: Ttp Following Florid Inflammatory Conditionsmentioning

confidence: 86%

Atypical presentations of thrombotic thrombocytopenic purpura: A review

Sarode

2008

J of Clinical Apheresis

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“…17,18 In this ELISA kit, the plate is coated with monoclonal anti-ADAMTS13 antibody that captures ADAMTS13 antigen in plasma. The ADAMTS13 antigen is subsequently detected by a second polyclonal rabbit antihuman ADAMTS13 antibody conjugated with horseradish peroxidase.…”

Section: Measurement Of Adamts13 Biomarkersmentioning

confidence: 99%

“…19 ADAMTS13 autoantibody (IgG) was quantified using a commercially available ELISA kit from American Diagnostica Inc. (IMUBIND® ADAMTS13 Autoantibody ELISA), as described previously. 17,20 …”

Section: Measurement Of Adamts13 Biomarkersmentioning

confidence: 99%

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome

Yang¹,

Jin²,

Lin³

et al. 2011

Haematologica

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BackgroundThe assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear. Design and MethodsIn this study, both ADAMTS13 antigen and activity levels were measured in 835 sequential samples from 40 consecutive patients who were followed for an average of 29 months throughout the course of acute episode plasma exchange treatment and clinical remission. ResultsDuring acute episodes, ADAMTS13 activity was severely deficient while ADAMTS13 antigen levels were more variable, ranging from severely deficient to as high as within the reference range. A severe depletion of ADAMTS13 antigen level during acute disease was, however, statistically associated with disease mortality (P=0.0322). For patients who achieved initial clinical responses, ADAMTS13 antigen levels appeared to be restored faster than ADAMTS13 activity to the normal range. Further analysis demonstrated that the ADAMTS13 antigen level at the time of initial clinical recovery was significantly higher in the patients who subsequently achieved a sustained clinical remission than in the group who soon after had an exacerbation (P=0.0187). ConclusionsOur data suggest that evaluation of ADAMTS13 antigen levels during the course of therapy and follow-up may offer additional useful information for the management of patients with thrombotic thrombocytopenic purpura.Key words: thrombotic thrombocytopenic purpura, ADAMTS13, prognosis, biomarker.Citation: Yang S, Jin M, Lin S, Cataland S, and Wu H. ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome Haematologica 2011;96(10)

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“…13;14 In some strains of mice complete ADAMTS13 deficiency was well tolerated, demonstrating that although ADAMTS13 deficiency caused a prothrombotic state, other genetic modifying factors or environmental triggers were necessary to induce abnormalities similar to TTP-HUS syndromes. 13,14 In the conditions that have been reported to trigger acute episodes of TTP-HUS, 5,[7][8][9][10][11][12] inflammatory cytokines may stimulate endothelial cell release of ultralarge and hyperreactive von Willebrand factor multimers and inhibit the cleavage of these multimers by ADAMTS13. 15 The pancreas is an organ frequently and severely involved in patients with TTP-HUS, 16,17 and pancreatic ischemia caused by TTP-HUS may contribute to the common symptom of abdominal pain.…”

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confidence: 99%