Thrombotic Thrombocytopenic Purpura: aetiology, pathophysiology and treatment

• Published in: Blood reviews Vol. 20; no. 1; pp. 51 - 60
• Main Authors: Murrin, R.J.A., Murray, J.A.
• Format: Journal Article
• Language: English
• Published: Kent Elsevier Ltd 2006; Elsevier
• Subjects: Aetiology; Anemias. Hemoglobinopathies; Biological and medical sciences; Diseases of red blood cells; Female; Hematologic and hematopoietic diseases; Humans; Male; Medical sciences; Pathophysiology; Plasmapheresis; Platelet diseases and coagulopathies; Purpura, Thrombotic Thrombocytopenic - etiology; Purpura, Thrombotic Thrombocytopenic - physiopathology; Purpura, Thrombotic Thrombocytopenic - therapy; Recurrence; Review; Thrombotic thrombocytopenic purpura; Treatment; von Willebrand Factor - physiology; Thrombotic thrombocytopenic purpura; Treatment; Review; Pathophysiology; Aetiology; Skin disease; Nervous system diseases; Urinary system disease; Hematology; Vascular disorders of the skin; Cardiovascular disease; Hemopathy; Vascular disease; Platelet; Hemolytic anemia; Etiology; Capillary vessel disease; Coagulopathy
• ISSN: 0268-960X; 1532-1681
• DOI: 10.1016/j.blre.2005.02.001

Thrombotic thrombocytopenic purpura (TTP) is a rare disorder whose varied clinical manifestations result from the formation of platelet-rich thrombi within the microvasculature and consequent tissue ischaemia. This review will outline how, in the eighty years since its initial description, scientific discoveries have not only led to a deeper understanding of the fundamental pathophysiology of TTP, but have also contributed to advances in the clinical management of this condition. Current research in this field will hopefully provide the basis for the design and development of novel therapeutic strategies.