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

被引:8
作者
Laemmle, Bernhard [1 ,2 ,3 ]
Vanhoorelbeke, Karen [4 ]
Hovinga, Johanna A. Kremer [2 ,3 ]
Knoebl, Paul [5 ]
机构
[1] Johannes Gutenberg Univ Mainz, Univ Med Ctr, Ctr Thrombosis & Hemostasis, Mainz, Germany
[2] Univ Bern, Bern Univ Hosp, Dept Hematol, Inselspital, Bern, Switzerland
[3] Univ Bern, Bern Univ Hosp, Cent Hematol Lab, Inselspital, Bern, Switzerland
[4] Katholieke Univ Leuven, Lab Thrombosis Res, Interdisciplinary Res Facil Life Sci, Campus Kulak Kortrijk, Kortrijk, Belgium
[5] Med Univ Vienna, Dept Med 1, Div Hematol & Hemostasis, Wahringer Gurtel 18-20, A-1090 Vienna, Austria
来源
HAMOSTASEOLOGIE | 2024年 / 44卷 / 01期
关键词
thrombotic thrombocytopenic purpura; ADAMS/ADAMTS; 13; management of disease; VON-WILLEBRAND-FACTOR; FACTOR-CLEAVING PROTEASE; THERAPEUTIC PLASMA-EXCHANGE; UPSHAW-SCHULMAN SYNDROME; SEVERE ADAMTS13 DEFICIENCY; HEMOLYTIC-UREMIC SYNDROME; FRENCH NATIONAL REGISTRY; FACTOR APTAMER ARC1779; FRESH-FROZEN PLASMA; RECOMBINANT ADAMTS13;
D O I
10.1055/a-2223-9484
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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.
引用
收藏
页码:59 / 73
页数:15
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