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Article ; Online: 100 Years of Thrombotic Thrombocytopenic Purpura: A Story of Death and Life.

Lämmle, Bernhard / Vanhoorelbeke, Karen / Kremer Hovinga, Johanna A / Knöbl, Paul

2024 Volume 44, Issue 1, Page(s) 59–73

Abstract: One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli Moschcowitz. In honor of this report, this article, written by distinguished specialists in ...

Abstract	One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli Moschcowitz. In honor of this report, this article, written by distinguished specialists in TTP, reviews the increase in scientific knowledge on this disease during the last 100 years. It covers the scientific progress from plasma therapy, the first beneficial treatment for TTP, to the elucidation of the pathophysiology, the discovery of ADAMTS13, the development of assays and targeted therapies up to the modern treatment concepts, that improved the outcome of TTP from an incurable disease to a well understood and treatable disorder.
MeSH term(s)	Humans ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/therapy ; ADAM Proteins ; ADAMTS13 Protein
Chemical Substances	ADAM Proteins (EC 3.4.24.-) ; ADAMTS13 Protein (EC 3.4.24.87)
Language	English
Publishing date	2024-02-28
Publishing country	Germany
Document type	Journal Article
ZDB-ID	801512-0
ISSN	2567-5761 ; 0720-9355
ISSN (online)	2567-5761
ISSN	0720-9355
DOI	10.1055/a-2223-9484
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Article: ADAMTS13 and Non-ADAMTS13 Biomarkers in Immune-Mediated Thrombotic Thrombocytopenic Purpura.

Bonnez, Quintijn / Sakai, Kazuya / Vanhoorelbeke, Karen

Journal of clinical medicine

2023 Volume 12, Issue 19

Abstract: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare medical emergency for which a correct and early diagnosis is essential. As a severe deficiency in A Disintegrin And Metalloproteinase with ThromboSpondin type 1 repeats, member 13 ( ... ...

Abstract	Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare medical emergency for which a correct and early diagnosis is essential. As a severe deficiency in A Disintegrin And Metalloproteinase with ThromboSpondin type 1 repeats, member 13 (ADAMTS13) is the underlying pathophysiology, diagnostic strategies require timely monitoring of ADAMTS13 parameters to differentiate TTP from alternative thrombotic microangiopathies (TMAs) and to guide initial patient management. Assays for conventional ADAMTS13 testing focus on the enzyme activity and presence of (inhibitory) anti-ADAMTS13 antibodies to discriminate immune-mediated TTP (iTTP) from congenital TTP and guide patient management. However, diagnosis of iTTP remains challenging when patients present borderline ADAMTS13 activity. Therefore, additional biomarkers would be helpful to support correct clinical judgment. Over the last few years, the evaluation of ADAMTS13 conformation has proven to be a valuable tool to confirm the diagnosis of acute iTTP when ADAMST13 activity is between 10 and 20%. Screening of ADAMTS13 conformation during long-term patient follow-up suggests it is a surrogate marker for undetectable antibodies. Moreover, some non-ADAMTS13 parameters gained notable interest in predicting disease outcome, proposing meticulous follow-up of iTTP patients. This review summarizes non-ADAMTS13 biomarkers for which inclusion in routine clinical testing could largely benefit differential diagnosis and follow-up of iTTP patients.
Language	English
Publishing date	2023-09-24
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2662592-1
ISSN	2077-0383
ISSN	2077-0383
DOI	10.3390/jcm12196169
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Article ; Online: Glycans of plasma ADAMTS13.

Vanhoorelbeke, Karen

Blood

2016 Volume 128, Issue 21, Page(s) 2485–2486

MeSH term(s)	Humans ; Polysaccharides ; Purpura, Thrombotic Thrombocytopenic/blood ; von Willebrand Factor
Chemical Substances	Polysaccharides ; von Willebrand Factor
Language	English
Publishing date	2016-11-05
Publishing country	United States
Document type	Journal Article ; Comment
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood-2016-10-738773
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Article: 100 Years of Thrombotic Thrombocytopenic Purpura: A Story of Death and Life

Lämmle, Bernhard / Vanhoorelbeke, Karen / Kremer Hovinga, Johanna A. / Knöbl, Paul

Hämostaseologie

2024 Volume 44, Issue 01, Page(s) 59–73

Abstract	One hundred years ago, in 1924, the first description of a patient with a disease, now known as thrombotic thrombocytopenic purpura (TTP) was published by Dr. Eli Moschcowitz. In honor of this report, this article, written by distinguished specialists in TTP, reviews the increase in scientific knowledge on this disease during the last 100 years. It covers the scientific progress from plasma therapy, the first beneficial treatment for TTP, to the elucidation of the pathophysiology, the discovery of ADAMTS13, the development of assays and targeted therapies up to the modern treatment concepts, that improved the outcome of TTP from an incurable disease to a well understood and treatable disorder.
Keywords	thrombotic thrombocytopenic purpura ; ADAMS/ADAMTS 13 ; management of disease
Language	English
Publishing date	2024-02-01
Publisher	Georg Thieme Verlag KG
Publishing place	Stuttgart ; New York
Document type	Article
ZDB-ID	801512-0
ISSN	2567-5761 ; 0720-9355
ISSN (online)	2567-5761
ISSN	0720-9355
DOI	10.1055/a-2223-9484
Database	Thieme publisher's database

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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
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Article ; Online: ADAMTS13 conformation is closed in non-immune acquired thrombotic thrombocytopenic purpura of unidentified pathophysiology.

Joly, Bérangère S / Roose, Elien / Coppo, Paul / Vanhoorelbeke, Karen / Veyradier, Agnès

Haematologica

2023 Volume 108, Issue 2, Page(s) 638–644

MeSH term(s)	Humans ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Autoantibodies ; ADAM Proteins ; ADAMTS13 Protein
Chemical Substances	Autoantibodies ; ADAM Proteins (EC 3.4.24.-) ; ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87)
Language	English
Publishing date	2023-02-01
Publishing country	Italy
Document type	Letter ; Research Support, Non-U.S. Gov't
ZDB-ID	2333-4
ISSN	1592-8721 ; 0017-6567 ; 0390-6078
ISSN (online)	1592-8721
ISSN	0017-6567 ; 0390-6078
DOI	10.3324/haematol.2022.280768
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Article ; Online: Insights into ADAMTS13 structure: impact on thrombotic thrombocytopenic purpura diagnosis and management.

Roose, Elien / Veyradier, Agnès / Vanhoorelbeke, Karen

Current opinion in hematology

2020 Volume 27, Issue 5, Page(s) 320–326

Abstract: Purpose of review: Fundamental knowledge on the role of a disintegrin and metalloprotease with thrombospondin type one repeats, member 13 (ADAMTS13) has been crucial to better understand the pathophysiology of the rare and life-threatening disease ... ...

Abstract	Purpose of review: Fundamental knowledge on the role of a disintegrin and metalloprotease with thrombospondin type one repeats, member 13 (ADAMTS13) has been crucial to better understand the pathophysiology of the rare and life-threatening disease thrombotic thrombocytopenic purpura (TTP). Recent findings: ADAMTS13 works through a molecular zipper mechanism to proteolyze its substrate von Willebrand factor (VWF). Recent insights into the structure and function of ADAMTS13 led to the identification of an allosteric activation mechanism. Therefore, ADAMTS13 is roughly folded in two in which the N-terminal spacer (S) domain and C-terminal T7-CUB2 domains interact to adopt a closed conformation. Upon substrate binding, ADAMTS13 adopts an open conformation in which the S-T7-CUB2 interaction is abrogated to further position VWF towards the catalytic cleft, inducing activation of the latent metalloprotease domain and resulting in cleavage of VWF. Unravelling the structure function relationship of ADAMTS13 helped identifying open ADAMTS13 as a novel and unique biomarker for immune-mediated TTP (iTTP). This novel biomarker has potential in the diagnosis, treatment and follow-up of iTTP. Summary: In this review, the most recent findings on the structure and working mechanism of ADAMTS13 are addressed. In addition, how those findings led to the identification of a novel biomarker, and how this novel biomarker could have an impact on the diagnosis, management and follow-up of iTTP patients are discussed.
MeSH term(s)	ADAMTS13 Protein/metabolism ; Female ; Humans ; Purpura, Thrombotic Thrombocytopenic/diagnosis ; Purpura, Thrombotic Thrombocytopenic/therapy
Chemical Substances	ADAMTS13 Protein (EC 3.4.24.87) ; ADAMTS13 protein, human (EC 3.4.24.87)
Language	English
Publishing date	2020-07-21
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	1153887-9
ISSN	1531-7048 ; 1065-6251
ISSN (online)	1531-7048
ISSN	1065-6251
DOI	10.1097/MOH.0000000000000602
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Article ; Online: Spatiotemporal profile of neutrophil extracellular trap formation in a mouse model of ischemic stroke.

De Wilde, Maaike / Desender, Linda / Tersteeg, Claudia / Vanhoorelbeke, Karen / De Meyer, Simon F

Research and practice in thrombosis and haemostasis

2022 Volume 7, Issue 1, Page(s) 100028

Abstract: Background: Thromboinflammatory processes modulate the complex pathophysiology of cerebral ischemia-reperfusion (I/R) injury in ischemic stroke, but the exact underlying mechanisms remain poorly understood. Emerging evidence indicates that neutrophil ... ...

Abstract	Background: Thromboinflammatory processes modulate the complex pathophysiology of cerebral ischemia-reperfusion (I/R) injury in ischemic stroke, but the exact underlying mechanisms remain poorly understood. Emerging evidence indicates that neutrophil extracellular traps (NETs) might play an important role in the thromboinflammatory cascade. In addition, the link between von Willebrand factor (VWF) and neutrophil recruitment in the ischemic brain might promote thromboinflammation, possibly by the formation of NETs. Objectives: To study NET formation in a murine model of cerebral I/R injury in ischemic stroke. Methods: The filament-induced transient middle cerebral artery occlusion model was used to induce 60 minutes of focal cerebral ischemia after which reperfusion was allowed. At different time points postischemia, NETs were identified in the ischemic mouse brain using quantitative immunofluorescence microscopy. Results: NETs could be identified in the ipsilateral brain hemisphere. Interestingly, NETs could already be detected at 6 hours poststroke. Their presence increased at 12 hours, was highest at 24 hours, and decreased again 48 hours postischemia. Remarkably, NETs were predominantly localized within the brain vasculature postischemia, suggesting that NETs play a role in secondary microthrombosis. Strikingly, NET formation was significantly decreased in VWF-deficient mice compared to littermate wild-type mice 24 hours postischemia, indicating a possible role for VWF in promoting NETosis in the ischemic brain. Conclusion: This study identified the spatiotemporal profile of NET formation in a mouse model of cerebral I/R injury in ischemic stroke. NETs, potentially in combination with VWF, might be attractive targets for the development of novel therapeutic strategies in ischemic stroke treatment.
Language	English
Publishing date	2022-12-23
Publishing country	United States
Document type	Journal Article
ISSN	2475-0379
ISSN (online)	2475-0379
DOI	10.1016/j.rpth.2022.100028
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Article ; Online: Toward gene therapy for congenital thrombotic thrombocytopenic purpura.

Dekimpe, Charlotte / Roose, Elien / Sakai, Kazuya / Tersteeg, Claudia / De Meyer, Simon F / Vanhoorelbeke, Karen

Journal of thrombosis and haemostasis : JTH

2022 Volume 21, Issue 5, Page(s) 1090–1099

Abstract: Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by a severe deficiency in the plasma metalloprotease ADAMTS-13. The current management of cTTP is dependent on the prophylactic administration of ADAMTS-13 via plasma infusion. This is a ... ...

Abstract	Congenital thrombotic thrombocytopenic purpura (cTTP) is caused by a severe deficiency in the plasma metalloprotease ADAMTS-13. The current management of cTTP is dependent on the prophylactic administration of ADAMTS-13 via plasma infusion. This is a demanding therapy for patients because transfusions are lifelong and time-consuming and allergic reactions frequently occur. Although current management of cTTP controls acute episodes, it does not provide a long-lasting cure for this disease. The endogenous expression of ADAMTS-13 after gene transfer would provide a curative therapy and ongoing research explores various preclinical gene therapeutic approaches for cTTP. This review focuses on the current state of the literature regarding preclinical gene therapy studies for cTTP and on the challenges of developing a gene therapy medicinal product for cTTP.
MeSH term(s)	Humans ; Purpura, Thrombotic Thrombocytopenic/genetics ; Purpura, Thrombotic Thrombocytopenic/therapy ; ADAMTS13 Protein ; Plasma ; Blood Transfusion ; Genetic Therapy/adverse effects
Chemical Substances	ADAMTS13 Protein (EC 3.4.24.87)
Language	English
Publishing date	2022-12-27
Publishing country	England
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2112661-6
ISSN	1538-7836 ; 1538-7933
ISSN (online)	1538-7836
ISSN	1538-7933
DOI	10.1016/j.jtha.2022.12.018
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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
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