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Plasma Exchange and N-Acetylcysteine Therapy in a Case of Congenital Thrombotic Thrombocytopenic Purpura Presenting With Acute Renal Failure.
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent thrombotic microangiopathy (TMA) episodes.
View Article and Find Full Text PDFThrombotic Thrombocytopenic Purpura Masked by Severe Preeclampsia: A Case Report.
Cureus
November 2024
Hematology and Oncology, King Saud Medical City, Riyadh, SAU.
Thrombotic thrombocytopenic purpura (TTP) is an exceptionally rare complication during pregnancy and even rarer when it coincides with severe preeclampsia in the same index pregnancy. We report the case of a 36-year-old female who presented with confusion at 38 weeks of gestation. Although her signs and symptoms strongly suggested severe preeclampsia, she was expected to make a full recovery after an emergency C-section.
View Article and Find Full Text PDFEffect of peripheral blood absolute monocyte count at admission on relapse-free survival in patients with idiopathic thrombotic thrombocytopenic purpura in remission.
Front Med (Lausanne)
December 2024
Department of Clinical Laboratory, the First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China.
Introduction: Peripheral monocytes have been reported to correlate with clinical outcomes in various types of malignancies. Previous reports have also shown that acute-phase thrombotic thrombocytopenic purpura (TTP) plasma could induce the activation of monocytes. However, the significance of peripheral blood absolute monocyte count (AMC) in idiopathic TTP remains an unanswered question.
View Article and Find Full Text PDFOutcomes in patients with thrombotic microangiopathy associated with a trigger following plasma exchange: A systematic literature review.
Transfus Apher Sci
December 2024
Alexion, AstraZeneca Rare Disease, 121 Seaport Blvd, Boston, MA 02210, USA. Electronic address:
Plasma exchange (PE) outcomes in patients with trigger-associated thrombotic microangiopathy (TMA) have not been comprehensively reviewed. Embase and MEDLINE® were searched on 03/14/2022 for English language articles published after 2007, alongside a congress materials search (2019-2022; PROSPERO: CRD42022325170). Studies with patients with trigger-associated TMA (excluding thrombotic thrombocytopenic purpura, 'typical' hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli, post-partum TMA, and TMAs with known genetic cause) who received PE or plasma infusion (PI) and reported treatment response (including measures), safety, patient-/caregiver-reported outcomes, or economic burden data were examined.
View Article and Find Full Text PDFA Case of Thrombotic Microangiopathy Secondary to Hypertensive Emergency: Presentation, Management, and Distinguishing Features.
Cureus
November 2024
Department of Medicine, Mercyhealth Graduate Medical Education (GME) Consortium, Rockford, USA.
Thrombotic microangiopathies (TMA) are a group of conditions that present with varying degrees of microthrombi, thrombocytopenia, microangiopathic hemolytic anemia, renal dysfunction, and neurological impairment. Etiologies can be primary, such as thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and atypical hemolytic uremic syndrome (aHUS), or secondary, such as due to systemic infections, malignancies, immune-mediated conditions, and hypertensive emergencies. In hypertensive emergencies, this presentation can occur from mechanical stress placed on red blood cells as they pass through narrowed arteries due to edema and microangiopathic changes within the vessels themselves.
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