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https://www.readbyqxmd.com/read/28428030/complement-inhibition-with-eculizumab-for-thrombotic-microangiopathy-rescues-a-living-donor-kidney-transplant-in-a-patient-with-antiphospholipid-antibody-syndrome
#1
Praveen Ramakrishnan Geethakumari, Patrick Mille, Rakesh Gulati, Srikanth Nagalla
Antiphospholipid antibody syndrome (APS) is an enigmatic heterogeneous disorder despite several revelations in its pathobiology. Renal transplantation in patients with APS has been notoriously difficult due to the high risk of development of thrombotic microangiopathy (TMA), which is often refractory to conventional treatment modalities such as aggressive anticoagulation and plasmapheresis. We describe a case of a 58-year-old male with secondary APS undergoing living unrelated renal transplantation for end-stage renal disease from lupus nephritis...
March 10, 2017: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/28424481/the-first-genome-wide-association-study-identifying-new-susceptibility-loci-for-obstetric-antiphospholipid-syndrome
#2
Mayumi Sugiura-Ogasawara, Yosuke Omae, Minae Kawashima, Licht Toyo-Oka, Seik-Soon Khor, Hiromi Sawai, Tetsuya Horita, Tatsuya Atsumi, Atsuko Murashima, Daisuke Fujita, Tomio Fujita, Shinji Morimoto, Eriko Morishita, Shinji Katsuragi, Tamao Kitaori, Kinue Katano, Yasuhiko Ozaki, Katsushi Tokunaga
Antiphospholipid syndrome (APS) is the most important treatable cause of recurrent pregnancy loss. The live birth rate is limited to only 70-80% in patients with APS undergoing established anticoagulant therapy. Lupus anticoagulant (LA), but not anticardiolipin antibody (aCL), was found to predict adverse pregnancy outcome. Recent genome-wide association studies (GWAS) of APS focusing on aCL have shown that several molecules may be involved. This is the first GWAS for obstetric APS focusing on LA. A GWAS was performed to compare 115 Japanese patients with obstetric APS, diagnosed according to criteria of the International Congress on APS, and 419 healthy individuals...
April 20, 2017: Journal of Human Genetics
https://www.readbyqxmd.com/read/28421915/use-of-a-modified-classic-bt-shunt-in-a-patient-with-multiple-conduit-obstructions-with-positive-lupus-anticoagulant
#3
Pallav Bhattarai, Ayesha Zia, Joseph M Forbess, Kristine J Guleserian, Susan R Hupp, Timothy Pirolli
The use of a right ventricle-to-pulmonary artery conduit has re-emerged as a popular alternative to a systemic artery-to-pulmonary artery shunt in the Norwood procedure. Both proximal obstruction secondary to dynamic compression and distal obstruction at the anastomosis site with the pulmonary arteries are well described. In an effort to prevent complications, a technique where in the conduit is placed through the entire full thickness, or dunked, through the RV free wall has been described. We report a case of a patient with HLHS and positive lupus anticoagulant who developed recurrent conduit obstructions...
January 1, 2017: World Journal for Pediatric & Congenital Heart Surgery
https://www.readbyqxmd.com/read/28420842/the-development-of-marked-collateral-circulation-due-to-inferior-vena-cava-filter-occlusion-in-a-patient-with-chronic-thromboembolic-pulmonary-hypertension-complicated-with-anti-phospholipid-syndrome
#4
Hajime Kasai, Nobuhiro Tanabe, Ken Koshikawa, Yasutaka Hirasawa, Toshihiko Sugiura, Seiichiro Sakao, Koichiro Tatsumi
A 30-year-old Japanese man was diagnosed with chronic thromboembolic pulmonary hypertension (CTEPH) with lupus anticoagulants (LAs) in 2003. He underwent pulmonary endarterectomy after the placement of an inferior vena cava filter (IVCF) in 2004, and treatment with warfarin was continued. In 2014, IVCF occlusion and marked collateral circulation were noted during an examination for transient dyspnea; however, his warfarin level was within the therapeutic range for 88.9% of the time from 2003 to 2014. We herein report a rare case of CTEPH and LAs with IVCF occlusion; in such cases, intense treatment may be required...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28411166/antiphospholipid-antibodies-can-identify-lupus-patients-at-risk-of-pulmonary-hypertension-a-systematic-review-and-meta-analysis
#5
REVIEW
Stéphane Zuily, Vinicius Domingues, Christine Suty-Selton, Valérie Eschwège, Laurent Bertoletti, Ari Chaouat, François Chabot, Véronique Regnault, Evelyn M Horn, Doruk Erkan, Denis Wahl
BACKGROUND: Pulmonary hypertension (PH) is a life-threatening condition that may affect outcomes in patients with systemic lupus erythematosus (SLE). The role of antiphospholipid antibodies (aPL) on the risk of PH is controversial. Therefore our objective was to estimate the risk of PH (WHO groups 1-5) including associated pulmonary arterial hypertension (APAH, WHO group 1 only) related to aPL in patients with SLE. METHODS: Systematic review and meta-analysis were performed: MEDLINE, EMBASE, Cochrane Library, congress abstracts, and reference lists of eligible studies were searched through 2015...
April 11, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28401801/thrombophilia-in-153-patients-with-premature-cardiovascular-disease-%C3%A2-age-45
#6
Alexander Milgrom, Kevin Lee, Matan Rothschild, Frini Makadia, Greg Duhon, Sarah Min, Ping Wang, Charles J Glueck
We assessed contributions of thrombophilia to premature cardiovascular disease (CVD) events (≤ age 45) in 153 patients. Test results of thrombophilia-hypofibrinolysis were obtained in 153 patients with CVD ≤ age 45, 110 healthy normal controls, and 110 patients who had venous thromboembolism (VTE) without CVD. Of the 153 patients with CVD, 121 (79%) had sustained myocardial infarction, 70 (46%) had coronary artery stenting, and 53 (35%) had coronary artery bypass grafts. The first CVD events occurred at ages >20 to 35 in 47 patients and at ages >35 to 45 in 106 patients...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28393620/progressively-worsening-premature-coronary-artery-disease-adding-anticoagulation-stabilizes-reverses-clinical-symptomatic-disease-progression-in-thrombophilic-atherothrombotic-patients-a-pilot-study
#7
Matan Rothschild, Vybhav Jetty, Christopher Mahida, Ping Wang, Marloe Prince, Naila Goldenberg, Charles J Glueck
In 35 patients with 116 severe premature cardiovascular disease (CVD) events (median age: 48 years), 14 having worsening CVD despite maximal intervention, we evaluated thrombophilia and speculated that anticoagulation might arrest-reverse progressive thrombophilic-atherothrombotic CVD. Thrombophilia-hypofibrinolysis in the 35 patients was compared to 110 patients with venous thromboembolism (VTE) without CVD and to 110 healthy normal controls. Efficacy-safety of anticoagulation was prospectively assessed in 14 of the 35 patients whose CVD worsened over 2 years despite maximal medical-surgical intervention...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28390037/clinical-usefulness-of-the-dilute-russell-viper-venom-time-test-for-patients-taking-warfarin
#8
Kazunori Kanouchi, Hiroto Narimatsu, Okio Ohnuma, Keita Morikane, Akira Fukao
Warfarin use often causes false-positive results in the dilute Russell viper venom time test (DRVVT). Thus, three sets of guidelines-those presented by the International Society on Haemostasis and Thrombosis (ISTH), the British Committee for Standards in Haematology (BCSH), and the Clinical and Laboratory Standards Institute (CLSI)-are advocated. We evaluated the clinical usefulness of the testing methods recommended in these three guidelines using laboratory samples. Of the 242 samples from patients using warfarin, 38 were positive for lupus anticoagulant (LA)...
April 7, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28389175/analytical-validation-of-a-multi-analyte-panel-with-cell-bound-complement-activation-products-for-systemic-lupus-erythematosus
#9
Thierry Dervieux, John Conklin, Jo-Anne Ligayon, Leilani Wolover, Tyler O'Malley, Roberta Vezza Alexander, Arthur Weinstein, Claudia A Ibarra
BACKGROUND: We describe the analytical validation of an assay panel intended to assist clinicians with the diagnosis of systemic lupus erythematosus (SLE). The multi-analyte panel includes quantitative assessment of complement activation and measurement of autoantibodies. METHODS: The levels of the complement split product C4d bound to erythrocytes (EC4d) and B-lymphocytes (BC4d) (expressed as mean fluorescence intensity [MFI]) are measured by quantitative flow cytometry, while autoantibodies (inclusive of antinuclear and anti-double stranded DNA antibodies) are determined by immunoassays...
April 4, 2017: Journal of Immunological Methods
https://www.readbyqxmd.com/read/28375199/stevens-johnson-syndrome-and-toxic-epidermal-necrolysis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-study
#10
Ana Paula Sakamoto, Clovis Artur Silva, Claudia Saad-Magalhães, Aline Nicácio Alencar, Rosa Maria Rodrigues Pereira, Katia Kozu, Cassia Maria Passarelli Lupoli Barbosa, Maria Teresa Terreri
OBJECTIVE: To assess Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) in a large population of childhood-onset systemic lupus erythematosus (cSLE) patients. METHODS: Multicenter study including 852 cSLE patients followed in Pediatric Rheumatology centers in São Paulo, Brazil. SJS was defined as epidermal detachment below 10% of body surface area (BSA), overlap SJS-TEN 10-30% and TEN greater than 30% of BSA. RESULTS: SJS and TEN was observed in 5/852 (0...
March 26, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28363116/clinical-significance-of-anti-domain-1-%C3%AE-2-glycoprotein-i-antibodies-in-antiphospholipid-syndrome
#11
Teresa Iwaniec, Marcin P Kaczor, Magdalena Celińska-Löwenhoff, Stanisław Polański, Jacek Musiał
BACKGROUND: Antiphospholipid syndrome (APS) is characterized by the presence of circulating antiphospholipid antibodies (aPL) in patients with thrombosis and/or pregnancy morbidity. In APS patients anti-domain 1 β2-glycoprotein I (anti-D1 β2GPI) IgG antibodies correlate strongly with thrombosis and to the lesser extent, with pregnancy complications. The aim of this study was to assess clinical utility of the anti-D1 β2GPI antibodies in the diagnosis and risk stratification of antiphospholipid syndrome...
February 24, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28361003/case-report-primary-osteonecrosis-associated-with-thrombophilia-hypofibrinolysis-and-worsened-by-testosterone-therapy
#12
Michael Ian Jarman, Kevin Lee, Ariel Kanevsky, Sarah Min, Ilana Schlam, Chris Mahida, Ali Huda, Alexander Milgrom, Naila Goldenberg, Charles J Glueck, Ping Wang
BACKGROUND: Familial and acquired thrombophilia are often etiologic for idiopathic hip and jaw osteonecrosis (ON), and testosterone therapy (TT) can interact with thrombophilia, worsening ON. CASE PRESENTATION: Case 1: A 62-year-old Caucasian male (previous deep venous thrombosis), on warfarin 1 year for atrial fibrillation (AF), had non-specific right hip-abdominal pain for 2 years. CT scan revealed bilateral femoral head ON without collapse. Coagulation studies revealed Factor V Leiden (FVL) heterozygosity, 4G/4G plasminogen activator inhibitor (PAI) homozygosity, high anti-cardiolipin (ACLA) IgM antibodies, and endothelial nitric oxide (NO) synthase (eNOS) T786C homozygosity (reduced conversion of L-arginine to NO, required for bone health)...
2017: BMC Hematology
https://www.readbyqxmd.com/read/28355988/direct-oral-anticoagulants-an-alternative-treatment-for-thrombotic-antiphospholipid-syndrome
#13
A S Resseguier, B Pereira, V Rieu, G Le Guenno, V Grobost, M Ruivard
Background Direct oral anticoagulants (DOACs) demonstrate a lower risk-benefit ratio than vitamin K antagonists (VKAs) for secondary thromboprophylaxis of thrombotic events. But there are no data on the efficacy of DOACs for the prevention of thrombotic recurrence in patients with antiphospholipid syndrome (APS). In this study, we evaluated the efficacy of DOACs to prevent recurrences of thrombotic events in patients with APS. Methods This was a single-center pilot, using a multi-step Fleming design. If seven or fewer patients presented treatment failure with rivaroxaban, the study could conclude efficacy...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28355984/hydroxychloroquine-induced-hyperpigmentation-in-systemic-diseases-prevalence-clinical-features-and-risk-factors-a-cross-sectional-study-of-41-cases
#14
E Bahloul, M Jallouli, S Garbaa, S Marzouk, A Masmoudi, H Turki, Z Bahloul
Introduction Hydroxychloroquine is an antimalarial agent widely prescribed in internal medicine, rheumatology and dermatology. Its use can be complicated by various side effects including skin pigmentation. Objectives The aim of the study is to review epidemiological, clinical features and risk factors of hydroxychloroquine-induced pigmentation. Materials and methods We performed a cross-sectional study conducted over a period of 5 months. During this period, patients who had been treated with hydroxychloroquine for over 6 months, in the internal medicine department, underwent a complete dermatological examination...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28341873/exploring-the-plasmatic-platelet-activating-factor-acetylhydrolase-activity-in-patients-with-anti-phospholipid-antibodies
#15
Martina Fabris, Adriana Cifù, Cinzia Pistis, Massimo Siega-Ducaton, Desrè Ethel Fontana, Roberta Giacomello, Elio Tonutti, Francesco Curcio
PURPOSE: To explore the role of plasmatic platelet-activating factor acetylhydrolase (PAF-AH), a marker of cardiovascular risk, in patients with anti-phospholipid antibodies (aPL). METHODS: PAF-AH activity was assessed in a series of 167 unselected patients screened for aPL in a context of thrombotic events, risk of thrombosis or obstetric complications and in 77 blood donors. RESULTS: 116/167 patients showed positive results for at least one aPL among IgG/IgM anti-prothrombin/phosphatidylserine (aPS/PT), anti-cardiolipin (aCL), anti-beta2-glycoprotein I (aβ2GPI) or lupus anticoagulant (LAC), while 51/167 patients resulted aPL-negative...
December 2017: Auto- Immunity Highlights
https://www.readbyqxmd.com/read/28339917/hellp-syndrome-as-a-potential-cause-of-false-positive-lupus-anticoagulant-testing
#16
Zhen Mei, Krzysztof Mikrut, Jonathan Miller
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28339897/reflex-lupus-anticoagulant-and-coagulation-factor-testing-has-low-utility-in-the-preoperative-surgical-hemostasis-assessment
#17
Stephen P Persaud, Ronald Jackups
No abstract text is available yet for this article.
March 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28321420/corrigendum-to-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
#18
(no author information available yet)
[This corrects the article DOI: 10.1177/2324709616661833.].
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28316135/variability-of-cut-off-values-for-the-detection-of-lupus-anticoagulants-results-of-an-international-multicenter-multiplatform-study
#19
A Tripodi, V Chantarangkul, M Cini, K Devreese, J S Dlott, R Giacomello, E Gray, C Legnani, M E Martinuzzo, P Pradella, A Siegemund, S Subramanian, P Suchon, S Testa
Essentials Between-lab variations of cut-off values in lupus anticoagulant detection are unknown. Cut-off values were calculated in 11 labs each testing plasma from 120 donors with 3 platforms. Major variation was observed even within the same platform. Cut-off values determined in different labs are not interchangeable. SUMMARY: Background Cut-off values for interpretation of lupus anticoagulant (LA) detection are poorly investigated. Aims (i) To assess whether results from healthy donors were normally distributed and (ii) the between-laboratories differences in cut-off values for screening, mixing and LA confirmation when calculated as 99th or 95th centiles, and (iii) to assess their impact on the detection rate for LA...
March 17, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28302338/-laboratory-diagnosis-of-antiphospholipid-syndrome-from-criteria-to-practice
#20
V Joste, M-A Dragon-Durey, L Darnige
The antiphospholipid syndrome is a bioclinical entity defined by thrombosis and/or obstetrical complications in the presence, at least 12 weeks apart, of antiphospholipid antibodies detected by coagulation test (lupus anticoagulant) or immunological assays (anticardiolipin, anti-β2-glycoprotein I antibodies). Biological markers' improvement such as anti-phosphatidylserine/prothrombin and biological score should allow better patients' management and preventive therapeutic for thrombosis and obstetrical complications...
March 14, 2017: La Revue de Médecine Interne
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