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This article presents a clinical case of ocular thrombotic microangiopathy of mixed origin (antiphospholipid syndrome, malignant arterial hypertension, multigenic thrombophilia). Multimodal imaging of the fundus provides a detailed assessment of its structures. Pathological changes in the choroid, the "retinal pigment epithelium - Bruch's membrane" complex, and the neurosensory retina, identified using fundus photography, short-wavelength autofluorescence, optical coherence tomography (OCT), and OCT angiography, are described as nonspecific in nature.
View Article and Find Full Text PDFIs the 99th Percentile Cutoff Still Relevant? A Single-Center Assessment of Different Thresholds for Diagnosing Antiphospholipid Syndrome.
J Appl Lab Med
December 2024
ARUP Institute for Clinical and Experimental Pathology, Salt Lake City, UT, United States.
Background: The 2023 American College of Rheumatology and modified Sapporo criteria for antiphospholipid syndrome (APS) recommend ELISA to detect anticardiolipin (aCL) and anti-β2-glycoprotein I (aβ2GP1) IgG/IgM antibodies, focusing on moderate to high levels or exceeding the 99th percentile. This study aims to establish the 99th percentile threshold for anti-phospholipid (aPL) antibodies and compare the diagnostic accuracies of these thresholds with manufacturer cutoffs using 2 methodologies.
Methods: The 99th percentile cutoffs for aPL antibodies from 305 healthy donors were established using Aptiva, Particle-Based Multi-Analyte Technology (PMAT), and QUANTA Lite (QL) ELISA, following nonparametric reference interval estimation.
Interpreting high levels of unfolded Von Willebrand Factor in patients with the antiphospholipid syndrome.
Front Immunol
December 2024
Coagulation Laboratory, Department of Laboratory Medicine, Ghent University Hospital, Ghent, Belgium.
Introduction: Unfolded Von Willebrand Factor (VWF) is increased in thrombotic pathologies such as myocardial infarction. Unfolded VWF mediates the binding of platelets without the need for collagen. β-glycoprotein I (β-GPI) is a natural inhibitor of the platelet-VWF interaction.
View Article and Find Full Text PDFPreceding Antiphospholipid Syndrome before the Onset of Systemic Lupus Erythematosus Presenting with Iliocaval Deep Vein Thrombosis: A Case Report and Literature Review.
Ann Vasc Dis
December 2024
Department of Vascular Surgery, Tokoname Municipal Hospital, Tokoname, Aichi, Japan.
Antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE) are similar to be characterized by thromboembolic events and various clinical manifestations. We experienced a 21-year-old man with acute iliocaval deep vein thrombosis (DVT). Primary APS was initially diagnosed on the criteria, and after multidisciplinary treatment, iliocaval DVT was gradually regressed.
View Article and Find Full Text PDFCase Report: Primary catastrophic antiphospholipid syndrome in a pediatric patient with cerebral venous sinus thrombosis as the first manifestation.
Front Pediatr
December 2024
Pediatric Intensive Care Unit, The Second Hospital of Lanzhou University, Lanzhou, China.
Background: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent vascular thrombotic events. Catastrophic APS (CAPS), which can result in multiple organ failure and even death, is the most severe manifestation of APS. Herein, we report the case of a pediatric patient with CAPS, including the clinical course, diagnosis, and treatment, with the goal of expanding the literature on this condition, as reports of CAPS in pediatric patients are rare.
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