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Hamostaseologie 2024; 44(01): 059-073
DOI: 10.1055/a-2223-9484
Review Article

100 Years of Thrombotic Thrombocytopenic Purpura: A Story of Death and Life

Bernhard Lämmle
1   Center for Thrombosis and Hemostasis, University Medical Center of the Johannes Gutenberg University, Mainz, Germany
2   Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
,
Karen Vanhoorelbeke
3   Laboratory for Thrombosis Research, Interdisciplinary Research Facility Life Sciences, Katholieke Universiteit Leuven Campus Kulak Kortrijk, Kortrijk, Belgium
,
Johanna A. Kremer Hovinga
2   Department of Hematology and Central Hematology Laboratory, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
,
Paul Knöbl
4   Department of Medicine 1, Division for Hematology and Hemostasis, Medical University of Vienna, Vienna, Austria
› Author Affiliations Funding None.
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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


Publication History

Received: 06 November 2023

Accepted: 06 December 2023

Article published online:
28 February 2024

© 2024. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 

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