Evidence map›Paper›PMID 25503977›Full record

ReviewThrombosis and haemostasis2015

VWF excess and ADAMTS13 deficiency: a unifying pathomechanism linking inflammation to thrombosis in DIC, malaria, and TTP.

Michael Schwameis, Christian Schörgenhofer, Alice Assinger, Margarete M Steiner, Bernd Jilma

Open access · greenAbstract readReview
In one paragraph

Review in Thrombosis and haemostasis, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 73 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
73citing papers in PubMed, 1 pooled it
4.0field-weighted citation impact, top 5% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

73 citing papers in PubMed, 1 synthesis or guideline pooled it, 136 citations in OpenAlex.

  1. Pooled it
  2. Trial
  3. Trial
  4. Article
  5. Review
  6. Review
  7. Article
  8. Review
  9. Article
  10. Review
  11. Review
  12. ADAMTS-13 Behavior in Thrombocytopenia of Infectious Origin in ICU Patients.Indian journal of critical care medicine : peer-reviewed, official publication of Indian Society of Critical Care Medicine · 2025
    Article
  13. Review
  14. Review
  15. The Highs and Lows of ADAMTS13 Activity.Journal of clinical medicine · 2024
    Review
  16. Article
  17. Review
  18. Recombinant ADAMTS-13 Improves Survival of Mice Subjected to Endotoxemia.International journal of molecular sciences · 2023
    Article
  19. Targeting the von Willebrand Factor-ADAMTS-13 axis in sickle cell disease.Journal of thrombosis and haemostasis : JTH · 2023
    Article
  20. Review

13 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

5 authors at 1 institution in 1 country.

Michael Schwameis
Christian Schörgenhofer
Alice Assinger
Margarete M Steiner
Bernd JilmaBernd Jilma, MD, Department of Clinical Pharmacology, Medical University of Vienna, Währinger Gürtel 18-20, A-1090 Vienna, Austria, Tel.: +43 1 40400 29810, Fax: +43 1 40400 29990, E-mail: bernd.jilma@meduniwien.ac.at.
Medical University of Vienna · AT

Funding

Austrian Science Fund FWF F 5404
6 · The paper itself

Abstract

Absent or severely diminished activity of ADAMTS13 (A Disintegrin And Metalloprotease with a ThromboSpondin type 1 motif, member 13) resulting in the intravascular persistence and accumulation of highly thrombogenic ultra large von Willebrand factor (UL-VWF) multimers is the pathophysiological mechanism underlying thrombotic thrombocytopenic purpura. Reduced VWF-cleaving protease levels, however, are not uniquely restricted to primary thrombotic microangiopathy (TMA), e. g. thrombotic thrombocytopenic purpura, but also occur in other life-threatening thrombocytopenic conditions: severely decreased ADAMTS13 activity is seen in severe sepsis, disseminated intravascular coagulation (DIC) and complicated malarial infection. The clinical relevance of these secondary thrombotic microangiopathies is increasingly recognised, but its therapeutic implications have not yet been determined. The presence of a secondary TMA in certain diseases may define patient groups which possibly could benefit from ADAMTS13 replacement or a VWF-targeting therapy. This short-review focuses on the role of UL-VWF multimers in secondary TMA and discusses the potential of investigational therapies as candidates for the treatment of TTP. In conclusion, prospective clinical trials on the effectiveness of protease replacementin vivo seem reasonable. Carefully selected patients with secondary TMA may benefit from therapies primarily intended for the use in patients with TTP.

Indexed as

ADAM ProteinsADAMTS13 ProteinAnimalsBiomarkersDisseminated Intravascular CoagulationEnzyme Replacement TherapyFibrinolytic AgentsHumansInflammationMalaria, FalciparumProtein MultimerizationPurpura, Thrombotic ThrombocytopenicThrombosisThrombotic MicroangiopathiesUp-Regulationvon Willebrand FactorADAM ProteinsADAMTS13 ProteinADAMTS13 protein, humanBiomarkersFibrinolytic Agentsvon Willebrand FactorADAMTS13DICmalariasepsisTTPVWF

Identifiers

PMID25503977
PMCPMC4745134
OpenAlexW2154205460

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.