The case of a woman with thrombotic thrombocytopenic purpura refractory to prolonged treatment with plasma exchange and steroid treatment is described. The addition of vincristine yielded a complete response, which has been maintained for 9 months up to the time of this report.
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http://dx.doi.org/10.1007/s002770050471 | DOI Listing |
Blood Adv
January 2025
KU Leuven Campus Kulak Kortrijk, Kortrijk, Belgium.
Allosteric regulation of ADAMTS13 (A Disintegrin And Metalloproteinase with ThromboSpondin type-1 motif, member 13) activity involves an interaction between its Spacer (S) and CUB1-2 domains to keep the enzyme in a closed, latent conformation. Monoclonal antibodies (mAb) uncouple the S-CUB interaction to open the ADAMTS13 conformation and thereby disrupt the global enzyme latency. The molecular mechanism behind this mAb-induced allostery remains poorly understood.
View Article and Find Full Text PDFClin Appl Thromb Hemost
January 2025
Department of Rheumatology, The First People's Hospital of Yunnan Province, The Affiliated Hospital of Kunming University of Science and Technology, Yunnan Province Clinical Research Center for Hematologic Disease, Kunming, Yunnan, China.
Objectives: To explore the risk factors for thrombi occurring in patients with immune thrombocytopenia (ITP) and establish a risk prediction model to better predict the risk of thrombosis in patients with ITP.
Methods: We retrospectively analyzed 350 ITP patients who had been hospitalized in the First People's Hospital of Yunnan Province between January 2024 and June 2024. For all patients, we recorded demographic characteristics and clinical data, analyzed the risk factors for thrombosis in ITP patients and then developed a risk prediction model.
Rev Med Chil
May 2024
Facultad de Medicina, Universidad Católica del Maule, Talca, Chile.
Early recognition of acute kidney injury is essential to prevent progression to chronic kidney disease. We present the case of a 19-year-old man with multiple emergency department visits for fatigue, abdominal pain, and intermittent dark urine. Upon admission, he had pancytopenia with elements suggestive of hemolysis and acute kidney injury.
View Article and Find Full Text PDFCureus
December 2024
Hematology and Oncology, University of Texas MD Anderson Cancer Center, Galveston, USA.
There are two main classifications for thrombotic thrombocytopenic purpura (TTP): immune and hereditary. The majority of TTP cases are immune in nature and are due to inhibitor autoantibodies against ADAMTS13. Hereditary TTP is caused by biallelic pathogenic variants in the ADAMTS13 gene.
View Article and Find Full Text PDFEur J Intern Med
January 2025
Laboratory of Pathology, Hebei Medical University, No. 361, Zhongshan Eastern Road, Shijiazhuang 050000, China. Electronic address:
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