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

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.
Competing Interests: PK: Grants or contracts from any entity: Sanofi/Ablynx, Novo Nordisk, Takeda, Roche. Consulting fees: Sanofi/Ablynx, Novo Nordisk, Takeda, Roche. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: Sanofi/Ablynx, Novo Nordisk, Takeda, Roche, Technoclone. Support for attending meetings and/or travel: Sanofi/Ablynx, Novo Nordisk, Takeda, Roche. Participation on a Data Safety Monitoring Board or Advisory Board: Sanofi/Ablynx, Novo Nordisk, Takeda.BL: Consulting fees: Hämatologie Praxis Bern, Monthly consultation and case discussions. Payment or honoraria for lectures, presentations, speakers bureaus, manuscript writing or educational events: Lecture fee, Long-term burden of TTP, Virtual meeting Takeda 18.05.2021; Lecture fee, History of ADAMTS13 and TTP, Educational meeting Alexion, Liverpool, 20.09.2023. Participation on a Data Safety Monitoring Board or Advisory Board: Chairman of DMCs for studies of recombinant ADAMTS13 in hereditary and acquired TTP, run by Takeda; Chairman of DMC of Mayari study, treatment of acquired TTP using caplacizumab and immunosuppression without plasma exchange, run by Sanofi.
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