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| http://dx.doi.org/10.1055/s-0040-1714272 | DOI Listing |
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GC1126A, a novel ADAMTS13 mutein, evades autoantibodies in immune-mediated thrombotic thrombocytopenic purpura.
Sci Rep
January 2025
Discovery3 Team, Department of Research and Early Development, GC Biopharma, 93, Ihyeon-ro 30Beon-gil, Giheung-gu, Yongin-si, Gyeonggi-do, South Korea.
Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare and life-threatening blood disorder characterized by the formation of blood clots in small blood vessels. It is caused by antibodies targeting the A disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13 (ADAMTS13), which plays a role in cleaving von Willebrand factor. Most patients with iTTP have autoantibodies against specific domains of the ADAMTS13 protein, particularly the cysteine-rich and spacer domains.
View Article and Find Full Text PDFIn the Era of Molecular Pathology, the Role of Morphological Changes in Megakaryocytes in Bone Marrow Aspiration in Cases of Isolated Thrombocytopenia.
Cureus
November 2024
Department of Pathology, Atal Bihari Vajpayee Government Medical College, Vidisha, IND.
Background: The most typical cause of thrombocytopenia is immune-mediated thrombocytopenic purpura (ITP). Thrombocytopenia can cause insufficient clot formation and increase the risk of bleeding. Bone marrow aspiration is commonly used for this purpose.
View Article and Find Full Text PDFRelapse-free survival is progressively shortened in a subset of Black patients with immune-mediated TTP treated in the rituximab era.
Blood Adv
January 2025
Department of Medicine, Johns Hopkins University, Baltimore, MD.
Article Synopsis
- - Immune thrombotic thrombocytopenic purpura (iTTP) is a serious condition involving low platelet counts due to a deficiency in the enzyme ADAMTS13, often treated with rituximab to prevent relapses.
- - A study using data from the USTMA registry found that the time without relapse (relapse-free survival or RFS) decreased after each rituximab treatment, particularly for Black patients, suggesting that the effectiveness of the drug diminishes with repeated use.
- - Both the USTMA registry and a separate cohort from Johns Hopkins and the University of Minnesota indicated that Black patients experience a significantly higher risk of relapse with subsequent rituximab treatments, implying a need
Bleeding disorder in a Holstein calf comparable to bovine neonatal pancytopenia.
Aust Vet J
November 2024
Rochester Veterinary Practice, Rochester, Victoria, Australia.
The clinical findings associated with a bleeding disorder, suspected to be an immune mediated pathogenesis comparable to bovine neonatal pancytopenia (BNP), in a 14-day-old Holstein calf are summarised. The clinical examination, clinical laboratory findings, treatment, postmortem findings and referral laboratory diagnostics are reported and discussed in relation to existing knowledge of bleeding disorders in cattle. Veterinary attention was required for a twin 14-day-old Holstein calf that was lethargic, weak and had pale mucous membranes.
View Article and Find Full Text PDFMain features of ischemic stroke in patients with acute immune-mediated thrombotic thrombocytopenic purpura.
Thromb Res
November 2024
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Milan, Italy; Università degli Studi di Milano, Department of Pathophysiology and Transplantation, Milan, Italy. Electronic address:
Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a microangiopathy often characterized by acute neurological involvement including ischemic stroke (IS). The characteristics of IS in iTTP remain largely unknown.
Aims: To evaluate the epidemiology, neuroimaging patterns and risk factors of IS in iTTP patients.
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