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  1. Article ; Online: Unusually high ADAMTS13 activity in a patient with congenital thrombotic thrombocytopenic purpura.

    Subhan, Maryam Owais / de Groot, Rens / Scully, Marie

    British journal of haematology

    2022  Volume 200, Issue 5, Page(s) 673–675

    MeSH term(s) Humans ; ADAMTS13 Protein ; Autoantibodies ; Purpura, Thrombotic Thrombocytopenic/diagnosis
    Chemical Substances ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87) ; Autoantibodies
    Language English
    Publishing date 2022-11-28
    Publishing country England
    Document type Case Reports ; Letter ; Research Support, Non-U.S. Gov't
    ZDB-ID 80077-6
    ISSN 1365-2141 ; 0007-1048
    ISSN (online) 1365-2141
    ISSN 0007-1048
    DOI 10.1111/bjh.18581
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Uh III Zs.182: Show issues Location:
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  2. Article ; Online: Peak ADAMTS13 activity to assess ADAMTS13 conformation and risk of relapse in immune-mediated Thrombotic Thrombocytopenic Purpura.

    Prasannan, Nithya / Dragunaite, Bertina / Subhan, Maryam Owais / Thomas, Mari / de Groot, Rens / Singh, Deepak / Vanhoorelbeke, Karen / Scully, Marie

    Blood

    2024  

    Abstract: Previous studies have demonstrated that more than 38% of immune-mediated thrombotic thrombocytopenic purpura (TTP) patients in remission with activity >50% had an open ADAMTS13 conformation. We assessed ADAMTS13 conformation in remission (ADAMTS13 ... ...

    Abstract Previous studies have demonstrated that more than 38% of immune-mediated thrombotic thrombocytopenic purpura (TTP) patients in remission with activity >50% had an open ADAMTS13 conformation. We assessed ADAMTS13 conformation in remission (ADAMTS13 activity >60%), focussing on peak ADAMTS13 activity levels and longitudinal assessment in 420 samples across 157 patients. Fewer cases had open conformation at peak ADAMTS13 activity compared to unselected remission samples with ADAMTS13 activity>60% (23% vs 43%). Patients with a closed ADAMTS13 conformation at peak ADAMTS13 activity had an 8-fold lower relapse rate in the subsequent year (9% vs 46%; OR 8.4, p=0.007) and a 5-fold lower relapse rate within 2 years (23% vs 62%; OR 5.3, p=0.02) compared to cases with an open conformation. Patients with an open conformation at peak ADAMTS13 activity required pre-emptive anti-CD20 treatment earlier than patients with a closed conformation (median 10 vs 25 months, p=0.02). Longitudinally, an open conformation was evident at, and often preceded relapse. Where the conformation was already open prior to relapse, an increase in conformation index at relapse was seen despite undetectable anti-ADAMTS13 IgG antibody. In cases with detectable anti-ADAMTS13 IgG antibody, these became undetectable prior to achieving closed conformation, highlighting the relapse risk even with undetectable anti-ADAMTS13 IgG antibody and the clinical utility of open/closed during monitoring. This is the first study to show an association between relapse risk and ADAMTS13 conformation when activity levels are at a peak. Open conformation identifies antibody mediated subclinical disease which is not detectable through current ADAMTS13 testing.
    Language English
    Publishing date 2024-03-19
    Publishing country United States
    Document type Journal Article
    ZDB-ID 80069-7
    ISSN 1528-0020 ; 0006-4971
    ISSN (online) 1528-0020
    ISSN 0006-4971
    DOI 10.1182/blood.2023023269
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.MO 364: Show issues

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  3. Article ; Online: ADAMTS13 activity testing: evaluation of commercial platforms for diagnosis and monitoring of thrombotic thrombocytopenic purpura.

    Singh, Deepak / Subhan, Maryam Owais / de Groot, Rens / Vanhoorelbeke, Karen / Zadvydaite, Almina / Dragūnaitė, Bertina / Scully, Marie

    Research and practice in thrombosis and haemostasis

    2023  Volume 7, Issue 2, Page(s) 100108

    Abstract: Background: ADAMTS13 activity is one of the key investigations needed to diagnose thrombotic thrombocytopenic purpura, and there are a number of different assays available to measure it. HemosIL AcuStar, a chemiluminescent immunoassay, was developed and ...

    Abstract Background: ADAMTS13 activity is one of the key investigations needed to diagnose thrombotic thrombocytopenic purpura, and there are a number of different assays available to measure it. HemosIL AcuStar, a chemiluminescent immunoassay, was developed and used as a quicker, automated test. In clinical practice, discrepancies between AcuStar and the gold standard FRETS-VWF73 have been documented in a manner that would affect diagnosis and treatment.
    Objectives: We aimed to identify and highlight clinical situations where this discrepancy occurs and to attempt to determine the cause.
    Method: Therefore, we undertook a study to compare the FRETS-VWF73 assay with AcuStar, the Technozym Activity ELISA, and Ceveron FRET assays using a mixture of 94 retrospective and prospective patient samples.
    Results: We found that although the concordance between FRETS-VWF73 and the other methods was generally very good, discrepancies were found in a small number tested on AcuStar affecting diagnosis (5 of 32) and follow-up (7 of 51). A Wilcoxon test comparing FRETS-VWF73 to the AcuStar results suggested that the AcuStar results were significantly lower in 42 samples tested on all 4 platforms. We investigated potential causes for this difference by testing the impact of high vWF levels and addition of a monoclonal ADAMTS13 autoantibody (3H9) to samples. We found no impact of high vWF levels on interassay variability but found that 3H9 reduced ADAMTS13 activity levels much more in AcuStar and ELISA assays than in FRETS assays.
    Conclusion: Based on our findings, we would suggest that when AcuStar is used upfront to guide management, a second testing method should be used in patients with an atypical thrombotic thrombocytopenic purpura presentation or unexpectedly slow ADAMTS13 recovery.
    Language English
    Publishing date 2023-03-10
    Publishing country United States
    Document type Journal Article
    ISSN 2475-0379
    ISSN (online) 2475-0379
    DOI 10.1016/j.rpth.2023.100108
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article ; Online: Alterations in B- and circulating T-follicular helper cell subsets in immune thrombotic thrombocytopenic purpura.

    Shin, Jin-Sup / Subhan, Maryam Owais / Cambridge, Geraldine / Guo, Yanping / de Groot, Rens / Scully, Marie / Thomas, Mari

    Blood advances

    2022  Volume 6, Issue 12, Page(s) 3792–3802

    Abstract: T follicular helper (Tfh) cells regulate development of antigen-specific B-cell immunity. We prospectively investigated B-cell and circulating Tfh (cTfh) cell subsets in 45 patients with immune thrombotic thrombocytopenic purpura (iTTP) at presentation ... ...

    Abstract T follicular helper (Tfh) cells regulate development of antigen-specific B-cell immunity. We prospectively investigated B-cell and circulating Tfh (cTfh) cell subsets in 45 patients with immune thrombotic thrombocytopenic purpura (iTTP) at presentation and longitudinally after rituximab (RTX). B-cell phenotype was altered at acute iTTP presentation with decreased transitional cells and post-germinal center (post-GC) memory B cells and increased plasmablasts compared with healthy controls. A higher percentage of plasmablasts was associated with higher anti-ADAMTS13 IgG and lower ADAMTS13 antigen levels. In asymptomatic patients with ADAMTS13 relapse, there were increased naïve B cells and a global decrease in memory subsets, with a trend to increased plasmablasts. Total circulating Tfh (CD4+CXCR5+) and PD1+ Tfh cells were decreased at iTTP presentation. CD80 expression was decreased on IgD+ memory cells and double-negative memory cells in acute iTTP. At repopulation after B-cell depletion in de novo iTTP, post-GC and double-negative memory B cells were reduced compared with pre-RTX. RTX did not cause alteration in cTfh cell frequency. The subsequent kinetics of naïve, transitional, memory B cells and plasmablasts did not differ significantly between patients who went on to relapse vs those who remained in remission. In summary, acute iTTP is characterized by dysregulation of B- and cTfh cell homeostasis with depletion of post-GC memory cells and cTfh cells and increased plasmablasts. Changes in CD80 expression on B cells further suggest altered interactions with T cells.
    MeSH term(s) B-Lymphocytes ; Humans ; Purpura, Thrombocytopenic, Idiopathic ; Purpura, Thrombotic Thrombocytopenic ; Receptors, CXCR5 ; Recurrence ; Rituximab/therapeutic use ; T Follicular Helper Cells ; T-Lymphocytes, Helper-Inducer
    Chemical Substances Receptors, CXCR5 ; Rituximab (4F4X42SYQ6)
    Language English
    Publishing date 2022-05-04
    Publishing country United States
    Document type Journal Article
    ZDB-ID 2915908-8
    ISSN 2473-9537 ; 2473-9529
    ISSN (online) 2473-9537
    ISSN 2473-9529
    DOI 10.1182/bloodadvances.2022007025
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    Zs.A 7153: Show issues Location:
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