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https://www.readbyqxmd.com/read/27913551/what-is-the-role-of-hydroxychloroquine-in-reducing-thrombotic-risk-in-patients-with-antiphospholipid-antibodies
#1
Tzu-Fei Wang, Wendy Lim
A 35-year-old man presents with an acute unprovoked deep vein thrombosis of the left lower extremity. He is treated with anticoagulation and elects to discontinue treatment after 6 months. He subsequently develops polyarthralgias, fatigue, and a malar rash, and a diagnosis of systemic lupus erythematosus is made based on laboratory and clinical findings. Additional laboratory testing reveals persistent triple positive antiphospholipid antibodies, including lupus anticoagulant, high titer anticardiolipin antibodies, and anti-β2-glycoprotein I antibodies...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27862892/lupus-anticoagulant-hypoprothrombinemia-syndrome-associated-with-severe-thrombocytopenia-in-a-child
#2
Aimee Foord, Nicole Baca, David Buchbinder, Arash Mahajerin
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS...
November 15, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#3
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27854095/cerebral-venous-sinus-thrombosis-in-a-patient-with-sj%C3%A3-gren-s-syndrome-with-atypical-antibodies-a-case-report
#4
Tsung-Han Ho, Yu-Wei Hsu, Chih-Wei Wang, Jiunn-Tay Lee, Chi-Hsin Ting, Fu-Chi Yang
BACKGROUND: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare. CASE REPORT: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait...
June 15, 2016: Acta Neurologica Taiwanica
https://www.readbyqxmd.com/read/27770664/clinical-implications-of-the-detection-of-antibodies-directed-against-domain-1-of-%C3%AE-2-glycoprotein-1-in-thrombotic-antiphospholipid-syndrome
#5
Silmara Montalvão, Priscila Soares Elídio, Sabrina da Silva Saraiva, Bruna de Moraes Mazetto, Marina Pereira Colella, Erich Vinícius de Paula, Simone Appenzeller, Joyce Annichino-Bizzacchi, Fernanda Andrade Orsi
INTRODUCTION: Antibodies directed against domain 1 of β2 glycoprotein 1 (aβ2GP1-Dm1) have been involved in the immunopathogenesis of antiphospholipid syndrome (APS). However, the clinical relevance of aβ2GP1-Dm1 in thrombotic APS has not yet been fully explored. OBJECTIVES: To determine the frequency of aβ2GP1-Dm1 in a cohort of patients with thrombotic APS, and to evaluate whether testing for aβ2GP1-Dm1 could have a clinical impact upon the risk assessment of the disease...
October 4, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27743753/lupus-anticoagulant-hypoprothrombinemia-syndrome-presenting-with-co-existing-cerebral-venous-thrombosis-and-subdural-hemorrhage
#6
N Bel Feki, S Zayet, I Ben Ghorbel, M-H Houman
The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count...
October 12, 2016: Journal des Maladies Vasculaires
https://www.readbyqxmd.com/read/27730530/a-combined-approach-using-global-coagulation-assays-quickly-differentiates-coagulation-disorders-with-prolonged-aptt-and-low-levels-of-fviii-activity
#7
Tomoko Matsumoto, Keiji Nogami, Midori Shima
Patients with mild/moderate hemophilia (H)A, acquired HA (AHA) and lupus anticoagulants (LA), have prolonged aPTTs with low levels of factor (F)VIII activity, but the differentiation of these disorders is complex and time consuming. We established an approach to quickly differentiate these disorders using comprehensive coagulation tests. Patients' plasmas with mild/moderate HA, AHA, LA without anti-phospholipid syndrome [LA-APS(-)], and LA with APS [LA-APS(+)] were examined using clot waveform analysis (CWA) and thrombin generation test (TGT)...
October 11, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27708976/the-clinical-significance-of-antiphospholipid-antibodies-in-systemic-lupus-erythematosus
#8
Ozan Ünlü, Stephane Zuily, Doruk Erkan
Antiphospholipid syndrome (APS) is the association of thrombosis and/or pregnancy morbidity with antiphospholipid antibodies (aPL). Thirty to forty percent of systemic lupus erythematosus (SLE) patients are tested positive for aPL, which may have an impact on the SLE presentation, management, and prognosis. Compared with SLE patients without aPL, those with aPL have a higher prevalence of thrombosis, pregnancy morbidity, valve disease, pulmonary hypertension, livedo reticularis, thrombocytopenia, hemolytic anemia, acute/chronic renal vascular lesions, and moderate/severe cognitive impairment; worse quality of life; and higher risk of organ damage...
June 2016: Eur J Rheumatol
https://www.readbyqxmd.com/read/27676644/novel-enzyme-immunoassay-system-for-simultaneous-detection-of-six-subclasses-of-antiphospholipid-antibodies-for-differential-diagnosis-of-antiphospholipid-syndrome
#9
Junzo Nojima, Yukari Motoki, Kazusa Hara, Toshiyuki Sakata, Kiyoshi Ichihara
Antiphospholipid syndrome, which often complicates systemic lupus erythematosus (SLE), features high occurrence of arterial and/or venous thrombosis and recurrent fetal loss. However, which antibody subclass contributes to which clinical event remains uncertain. We newly developed an up-to-date enzyme immunoassay system using the AcuStar automated analyzer (Instrumentation Laboratory, Bedford, Massachusetts, USA) for parallel detection of six subclasses of antiphospholipid antibodies (aPLs): anticardiolipin antibodies (aCL) of IgG, IgM, and IgA and anti-β2-glycoprotein I antibodies (aβ2GPI) of IgG, IgM, and IgA...
September 24, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/27639837/catastrophic-antiphospholipid-syndrome-caps-descriptive-analysis-of-500-patients-from-the-international-caps-registry
#10
Ignasi Rodríguez-Pintó, Marta Moitinho, Irene Santacreu, Yehuda Shoenfeld, Doruk Erkan, Gerard Espinosa, Ricard Cervera
OBJECTIVE: To analyze the clinical and immunologic manifestations of patients with catastrophic antiphospholipid syndrome (CAPS) from the "CAPS Registry". METHODS: The demographic, clinical and serological features of 500 patients included in the website-based "CAPS Registry" were analyzed. Frequency distribution and measures of central tendency were used to describe the cohort. Comparison between groups regarding qualitative variables was undertaken by chi-square or Fisher exact test while T-test for independent variables was used to compare groups regarding continuous variables...
September 15, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27638930/annals-express-antiphospholipid-and-antioangiogenic-activity-in-women-with-recurrent-miscarriage-and-antiphospholipid-syndrome
#11
H Fabián Pelusa, Eleonora Pezzarini, Cecilia Basiglio, Jorge Musuruana, Mariela Bearzotti, María José Svetaz, Stella Maris Daniele, Hebe Bottai, Sandra M M Arriaga
BACKGROUND: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by thrombosis, fetal losses and thrombocytopenia associated to antiphospholipid (APL) antibodies (Abs). They are directed to phospholipids, such as cardiolipins (a-cardiolipin, ACA) and lupus anticoagulant (LA), or to complexes formed by phospholipids and protein cofactors, such as β2 glycoprotein 1 (a-β2GP1) and annexin V (a-annexin V). These auto Abs may be considered as a family of Abs involved in thrombotic events and APL activity...
September 16, 2016: Annals of Clinical Biochemistry
https://www.readbyqxmd.com/read/27638140/pathogenesis-of-the-antiphospholipid-syndrome-revisited-time-to-challenge-the-dogma-comment
#12
V Pengo
I read with interest the paper by Lackner and Muller-Calleja recently published in the Journal [1]. I would like to comment on the issue concerning clinical studies. The authors stress the concept that 'Lupus Anticoagulant (LAC) is the antiphospholipid (aPL) test most consistently and strongly associated with thromboembolic events'. This statement is correct but the authors do not underline that the antibodies determining LAC activity and its association with thromboembolic events are those directed to β2-glycoprotein I (β2GPI) [2,3]...
September 16, 2016: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/27617946/a-case-of-pachydermodactyly-in-a-seventeen-year-old-associated-with-repetitive-minor-trauma
#13
W Abdelrahman, M Y Walsh, A Smyth, D Alderice
A boy presented initially to a Rheumatology clinic with a three year history of asymptomatic swelling of the third to fourth proximal interphalangeal (PIP) joints bilaterally. A presumptive diagnosis of seronegative arthritis was made. Sulfasalazine was commenced without improvement and resulted in mood disturbance. Blood tests including ESR, lupus anticoagulant, rheumatoid factor and CCP antibodies were unremarkable. Hand radiographs were normal. MRI showed oedema within soft tissues around PIP joints. His care was transferred to the Rheumatology unit in our hospital and the rheumatological diagnosis was revised; sulfasalazine was stopped and skin biopsy organised...
2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/27610369/clinical-application-of-revised-laboratory-classification-criteria-for-antiphospholipid-antibody-syndrome-is-the-follow-up-interval-of-12-weeks-instead-of-6-weeks-significantly-useful
#14
Sang Hyuk Park, Seongsoo Jang, Chan-Jeoung Park, Hyun-Sook Chi
Background. According to revised classification criteria of true antiphospholipid antibody syndrome, at least one of three antiphospholipid antibodies should be present on two or more occasions at least 12 weeks apart. However, it can be inconvenient to perform follow-up tests with interval of 12 weeks. We investigated clinical application of follow-up tests with interval of 12 weeks. Method. Totals of 67, 199, and 332 patients tested positive initially for the lupus anticoagulants confirm, the anti-β 2 glycoprotein-I antibody, and the anti-cardiolipin antibody test, respectively, from Jan 2007 to Jul 2009...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27549418/antiphospholipid-antibodies-among-pregnant-women-with-recurrent-fetal-wastage-in-a-tertiary-hospital-in-northern-nigeria
#15
Zubaida Garba Abdullahi, Mohammmed A Abdul, Sirajo M Aminu, Bolanle O P Musa, Lawal Amadu, El-Bashir M Jibril
CONTEXT: The association between antiphospholipid antibodies (APAs) and pregnancy loss has been established and now considered as a treatable cause of pregnancy loss. Data on the prevalence of APA in patients with recurrent pregnancy loss are scarce in our environment. AIMS: To determine the prevalence of APA in pregnant women with and without recurrent fetal wastage. SETTINGS AND DESIGN: Antenatal clinic of Ahmadu Bello University Teaching Hospital, Zaria, Nigeria...
July 2016: Annals of African Medicine
https://www.readbyqxmd.com/read/27539195/anti-mullerian-hormone-levels-decline-with-the-presence-of-antiphospholipid-antibodies
#16
Mario Vega, David H Barad, Yao Yu, Sarah K Darmon, Andrea Weghofer, Vitaly A Kushnir, Norbert Gleicher
PROBLEM: Autoimmunity is thought to be an important cause of premature ovarian senescence, characterized by abnormal ovarian reserve markers. Anti-Mullerian hormone (AMH) has emerged as the most reliable marker for ovarian reserve. We here investigated whether non-specific immune markers are associated with a low age-specific AMH. METHOD OF STUDY: To test the hypothesis that autoimmunity may predispose to low AMH, we investigated in 351 female infertile patients whether panels positive for non-specific immune tests (antinuclear antibody, antiphospholipid antibodies [APAs], lupus anticoagulant, antithyroid antibodies, and total immunoglobulin levels) are associated with low AMH levels...
October 2016: American Journal of Reproductive Immunology: AJRI
https://www.readbyqxmd.com/read/27536705/four-thrombotic-events-over-5-years-two-pulmonary-emboli-and-two-deep-venous-thrombosis-when-testosterone-hcg-therapy-was-continued-despite-concurrent-anticoagulation-in-a-55-year-old-man-with-lupus-anticoagulant
#17
Charles J Glueck, Kevin Lee, Marloe Prince, Vybhav Jetty, Parth Shah, Ping Wang
BACKGROUND: When exogenous testosterone or treatments to elevate testosterone (human chorionic gonadotropin [HCG] or Clomid) are prescribed for men who have antecedent thrombophilia, deep venous thrombosis and pulmonary embolism often occur and may recur despite adequate anticoagulation if testosterone therapy is continued. CASE PRESENTATION: A 55-year-old white male was referred to us because of 4 thrombotic events, 3 despite adequate anticoagulation over a 5-year period...
July 2016: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/27474775/rivaroxaban-therapy-false-positive-lupus-anticoagulant-screening-results-and-confirmatory-assay-results
#18
Lauren M Murer, Samuel J Pirruccello, Scott A Koepsell
OBJECTIVE: To examine the effects of patients taking the direct blood coagulation factor Xa inhibitor rivaroxaban on lupus anticoagulant testing results in a clinical setting. METHODS: We reviewed the results of lupus anticoagulant testing performed over a 2-year period. Of 59 patients who met criteria for a lupus anticoagulant, 18 were taking rivaroxaban. We reviewed and compared the parameters of lupus anticoagulant testing. RESULTS: The average dilute Russell viper venom time (DRVVT) and normal plasma-mix screening results to confirmation ratios in rivaroxaban-naïve patients were 1...
November 2016: Laboratory Medicine
https://www.readbyqxmd.com/read/27473743/prozone-effect-in-the-diagnosis-of-lupus-anticoagulant-for-the-lupus-anticoagulant-hypoprothrombinemia-syndrome
#19
Jing Jin, James L Zehnder
OBJECTIVES: The main clinical sequela of a lupus anticoagulant is increased thrombosis risk. However, bleeding due to lupus anticoagulant-hypoprothrombinemia syndrome is a rare but well-described manifestation of antiphospholipid syndrome. The association of acute acquired hypoprothrombinemia is caused by a lupus anticoagulant's specificity to prothrombin, which results in clearance of prothrombin and bleeding due to hypoprothrombinemia (usually <10% of normal). Severe life-threatening bleeding is most frequently reported in children with systemic lupus erythematosus or in healthy children after viral infection...
August 2016: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/27429595/thrombotic-risk-assessment-in-antiphospholipid-syndrome-the-role-of-new-antibody-specificities-and-thrombin-generation-assay
#20
REVIEW
Savino Sciascia, Simone Baldovino, Karen Schreiber, Laura Solfietti, Massimo Radin, Maria J Cuadrado, Elisa Menegatti, Doruk Erkan, Dario Roccatello
Antiphospholipid syndrome (APS) is an autoimmune condition characterized by the presence of antiphospholipid antibodies (aPL) in subjects presenting with thrombosis and/or pregnancy loss. The currently used classification criteria were updated in the international consensus held in Sidney in 2005. Vascular events seem to result of local procoagulative alterations upon triggers influence (the so called "second-hit theory"), while placental thrombosis and complement activation seem to lead to pregnancy morbidity...
2016: Clinical and Molecular Allergy: CMA
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