Thrombotic thrombocytopenic purpura (TTP) was first described by Eli Moschcowitz in 1924. The pathophysiology of this disease is related to unusual, large multimers of von Willebrand factor in microcirculation, that are insufficiently cleaved by ADAMTS13 protease (a disintegrin-like and metalloprotease with thrombospondin type 1motif,13). Congenital TTP/Upshaw-Schulman syndrome is less frequent than acquired one TTP/HUS (haemolytic-ureamic syndrome). Short characteristic of patients with inherited form of TTP is reported as well as their clinical and laboratory features and management of treatment.
Download full-text PDF |
Source |
|---|
Publication Analysis
Top Keywords
Similar Publications
More on the pathogenesis of thrombotic thrombocytopenic purpura. Comment on "Acquired thrombotic thrombocytopenic purpura without detectable anti-ADAMTS13 antibodies: a possible underlying autoimmune mechanism" and on "The different faces of thrombotic thrombocytopenic purpura".
Haematologica
January 2025
Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy; Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan.
Not available.
View Article and Find Full Text PDFInfective endocarditis causing acute aortic occlusion in a patient with systemic lupus erythematosus: A rare case report.
Int J Surg Case Rep
January 2025
Department of Vascular Surgery, Royal Perth Hospital, Perth 6000, Australia; University of Western Australia, School of Surgery, Perth 6000, Australia. Electronic address:
Introduction: We present a unique case of acute aortic occlusion secondary to infective endocarditis (IE).
Presentation Of Case: An Aboriginal Australian woman with systemic lupus erythematosus presented with fever, confusion, tachycardia, and tachypnoea and had cold, pulseless, insensate, and paralysed lower limbs. Computed tomography angiography revealed multifocal occlusion of the distal aorta and lower limb vessels.
COVID-19 associated thrombotic thrombocytopenic purpura: a case report and review of the literature.
Expert Rev Hematol
January 2025
Division of Hematology and Oncology, Naef K. Basile Cancer Institute, American University of Beirut Medical Center, Beirut, Lebanon.
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially life-threatening complication whose association with COVID-19 is controversial. Understanding this connection is essential due to its significant impact on patient outcomes, and timely diagnosis and intervention are critical in managing this condition effectively.
Areas Covered: This paper presents a case of TTP triggered by COVID-19 infection in a 48-year-old female.
UV immunothrombotic trombocytopenic purpura.
Transfus Apher Sci
January 2025
Banco de Sangre y Tejidos de Navarra, Navarre Health Service (SNS-O), Pamplona, Spain; Grupo Español de Aféresis (GEA)(Spanish Group of Apheresis), Spain. Electronic address:
A compound heterozygous mutation causes congenital thrombotic thrombocytopenic purpura: a case report.
Front Med (Lausanne)
January 2025
Department of Hematology, The Second Affiliated Hospital of Nanchang University, Nanchang, China.
Congenital thrombotic thrombocytopenic purpura (cTTP) is a thrombotic microangiopathy (TMA) characterized by severe hereditary ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13) deficiency caused by mutations. This rare autosomal recessive genetic disorder is often misdiagnosed as immune thrombocytopenia (ITP) or hemolytic uremic syndrome (HUS). Here, we report a 21-year-old male cTTP patient with a compound heterozygous mutation.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!