The antiphospholipid antibody syndrome (Lupus anticoagulans syndrome) is a rare form of coagulopathy due to the presence of autoantibodies against phospholipids or phospholipid-binding protein cofactors that can lead to vascular thrombosis. We report the case of a 57-year-old female patient presenting with decompensated duodenal stenosis due to a pancreatic tumor. Perioperative testing of coagulation markers revealed with 26 % a strongly decreased Quick-Test and with 81.4 s a prolonged partial thromboplastin time that persisted despite intravenous application of 80 mg vitamin K (Konakion) and 10 units of fresh frozen plasma. Subsequent screening for common causes of thrombophilia revealed antiphospholipid antibodies. Consequently, low molecular weight heparin (Dalteparin-Natrium) was administered perioperatively while a gastroenterostomy with entero-enterostomy was performed with uneventful postoperative course. With this presentation and an analysis of the contemporary literature we would like to discuss different aspects of Lupus anticoagulans syndrome.
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http://dx.doi.org/10.1055/s-2005-836796 | DOI Listing |
Inn Med (Heidelb)
April 2024
Medizinische Klinik VI Altersmedizin, Uniklinik RWTH Aachen Standort Franziskus, Morillenhang 27, 52074, Aachen, Deutschland.
A 73-year-old man with dementia was referred to our clinic with hypernatremia and volume depletion. New-onset neurogenic dysphagia was likely the reason for both. The patient had chronic embolic strokes on the computed tomography (CT) images.
View Article and Find Full Text PDFInt J Mol Sci
August 2022
Department of Hemato-Oncology, Faculty of Medicine and Dentistry, University Hospital Olomouc, Palacky University Olomouc, 77900 Olomouc, Czech Republic.
Antiphospholipid syndrome (APS) is a hypercoagulable state accompanied by the presence of heterogeneous antiphospholipid antibodies (aPL), which nonspecifically affect hemostasis by the presence of lupus anticoagulans (LA), anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein-I (anti-β2GPI), but also non-criteria antibodies such as antibodies against β2-glycoprotein-I domain I (anti-DI), anti-phosphatidylserine/prothrombin (anti-PS/PT), anti-annexin V, and many others. The main target of the antibodies is the activated protein C (APC) system, the elimination of which can manifest itself as a thrombotic complication. The aim of this study was to determine the thrombogenicity of antibodies using a modified protein C-activated thrombin generation assay (TGA) on a group of 175 samples suspected of APS.
View Article and Find Full Text PDFRheumatol Adv Pract
November 2021
Department of Hematology.
Objectives: Lupus anticoagulans (LACs) and aPLs, both further summarized as aPL, are frequently assessed in routine daily clinical practice in diagnostic workups for suspected autoimmune diseases or to test for underlying risk factors in patients with thrombosis or obstetric complications. The aim of this study was to determine the prevalence of aPL positivity in patients with an indication for aPL testing in routine clinical practice.
Methods: In this retrospective single-centre study, indication for aPL testing, aPL test results and clinical data were collected for patients tested between June 2015 and April 2018.
Hematology
December 2019
a Department of Medicine , Padova University School of Medicine, Padova , Italy.
Objectives: The relevance of detecting antibodies against anticardiolipin, β2-glycoprotein I (β2gpI) or lupus anticoagulant (LA), collectively called antiphospholipid autoantibodies (APA), in subjects with immune thrombocytopenia (ITP) is still a debated issue. In particular, whether APA profile may affect the clinical course of ITP is unknown.
Methods: In this study, we report our experience in a cohort of ITP patients with APA with specific interest to the relevance of different antiphospholipid antibody profiles in clinical outcome and response to treatment.
J Dermatolog Treat
September 2017
a Department of Dermatology , University Hospital of Münster, Münster , Germany.
Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disease characterized by arterial and/or venous thrombosis, recurrent abortions and detection of antiphospholipid antibodies. In fulminant cases, involvement of multiple organs can lead to significant morbidity and even fatal outcomes, so that a rapid, interdisciplinary treatment is needed. Here, we describe the case of a 39-year-old woman with a severe hard-to-treat APS with arterial occlusion and progressive skin necrosis, who was successfully treated with a combination therapy with plasmapheresis and rituximab.
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