In the 100 years since Dr. Eli Moschcowitz reported the first case of thrombotic thrombocytopenic purpura (TTP), there has been remarkable awareness and progress in the diagnosis and management of this rare blood disorder. This progress initially was the result of careful clinical observations followed by well thought-out therapeutic interventions, with dual goals of both improving outcomes and discerning the pathophysiology of TTP. The discovery of the ADAMTS13 protease set in motion efforts to more accurately define the specific etiologies of thrombotic microangiopathies (TMA) based on objective, scientific data rather than clinical characterizations alone. This accurate differentiation led to better and more revealing clinical trials and advancements in the treatment of TTP and other TMA. Further advances followed and included improvements in immune suppressive therapy and targeted therapies of immune-mediated TTP (iTTP) (caplacizumab) and congenital TTP (cTTP) (recombinant ADAMTS13). The longitudinal study of TTP patients revealed the unexpected risk for long-term complications in both iTTP and cTTP patients in remission. Ongoing studies aim to further understand the prevalence, mechanisms, and appropriate screening for these mood disorders, neurocognitive deficits, and cardiovascular complications that develop at remarkably high rates, and are associated with a decreased life expectancy. These discoveries are a result of the collaborative efforts of investigators worldwide that have been fostered by the frequent interactions of investigators via the International TTP Working Group meetings and TMA workshops held regularly at international meetings. These efforts will support the rapid pace of discovery and improved understanding of this rare disease.
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Review Article|
July 3, 2024
Thrombotic Thrombocytopenic Purpura: 100 Years of Research on Moschcowitz's Syndrome
Spero R. Cataland,
Ohio State University, Columbus, Ohio, United States
* Corresponding Author; email: spero.cataland@osumc.edu
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Paul Coppo,
Paul Coppo
Hopital Saint-Antoine, Paris, France
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Marie Scully, MBE,
Marie Scully, MBE
Department of Haematology, University College London Hospital, London, United Kingdom
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Bernhard Lämmle
Bernhard Lämmle
University Medical Center Mainz, Bolligen, Switzerland
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Blood blood.2023022277.
Article history
Submitted:
April 5, 2024
Revision Received:
June 21, 2024
Accepted:
June 23, 2024
Citation
Spero R. Cataland, Paul Coppo, Marie Scully, Bernhard Lämmle; Thrombotic Thrombocytopenic Purpura: 100 Years of Research on Moschcowitz's Syndrome. Blood 2024; blood.2023022277. doi: https://doi.org/10.1182/blood.2023022277
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