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Updated: Jun 13, 2026
09:42
A Fibrin-Enriched and tPA-Sensitive Photothrombotic Stroke Model
Published on: June 4, 2021
[Thrombotic thrombocytopenic purpura]
Javier de la Rubia1, Enric Contreras, Julio Del Río-Garma
1Servicio de Hematología, Hospital La Fe, Valencia, España. delarubia_jav@gva.es
Medicina Clinica
|April 27, 2010
Summary
Thrombotic thrombocytopenic purpura (TTP) is a dangerous platelet disorder. Deficiencies in ADAMTS13 enzyme cause TTP, necessitating new treatments due to frequent relapses.
Area of Science:
- Hematology
- Vascular Biology
- Enzymology
Context:
- Thrombotic thrombocytopenic purpura (TTP) historically presented with near 100% mortality and obscure etiology.
- Plasma exchange therapy, introduced in the late 1970s/early 1980s, significantly improved outcomes.
- The discovery of ADAMTS13 metalloprotease revolutionized understanding of TTP pathophysiology.
Purpose:
- To elucidate the role of ADAMTS13 in regulating von Willebrand factor size.
- To explain how ADAMTS13 deficiencies lead to microvascular thrombosis and end-organ ischemia in TTP.
- To highlight the impact of plasma exchange and the need for alternative therapies due to TTP relapses.
Summary:
- TTP is characterized by intravascular platelet clumping, often due to inherited or acquired deficiencies of the ADAMTS13 enzyme.
- ADAMTS13 is crucial for cleaving von Willebrand factor; its deficiency results in platelet-rich thrombi formation in microcirculation.
- Plasma exchange has been a successful treatment for TTP, but increasing relapse rates necessitate novel therapeutic strategies.
Impact:
- Plasma exchange has dramatically improved survival rates for adult TTP patients.
- Understanding ADAMTS13's role provides a molecular basis for TTP pathogenesis.
- The high frequency of TTP relapses underscores the urgent need for developing new therapeutic alternatives.
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