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https://www.readbyqxmd.com/read/28627093/characterization-of-thrombosis-in-patients-with-proteus-syndrome
#1
Kim M Keppler-Noreuil, Jay N Lozier, Julie C Sapp, Leslie G Biesecker
Patients with overgrowth and complex vascular malformation syndromes, including Proteus syndrome have an increased risk of thromboembolism. Proteus syndrome is a mosaic, progressive overgrowth disorder involving vasculature, skin, and skeleton, and caused by a somatic activating mutation in AKT1. We conducted a comprehensive review of the medical histories and hematologic evaluations of 57 patients with Proteus syndrome to identify potential risk factors for thrombosis. We found that six of ten patients, who were deceased, died secondary to deep venous thrombosis and/or pulmonary embolism...
June 19, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28603894/a-performance-evaluation-of-a-novel-human-recombinant-tissue-factor-prothrombin-time-reagent-revohem-%C3%A2-pt
#2
C Gardiner, K Kohama, I Patel, P Lane, S Dwyer, S J Machin, I J Mackie
INTRODUCTION: A new prothrombin time reagent (Revohem™ PT) based on recombinant human tissue factor produced by the silkworm-baculovirus expression system was tested. The aim of this study was to compare the performance of the new PT reagent with two widely used routine PT reagents. METHODS: All testing was performed on a Sysmex CS-5100 coagulometer. Revohem(™) PT was tested for imprecision and stability using normal and abnormal lyophilized commercial control plasmas...
June 12, 2017: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/28602126/clinical-and-prognostic-significance-of-lupus-anticoagulant-measurement-in-patients-with-lung-cancer
#3
Xianming Fei, Huan Wang, Lei Jiang, Tongwei Zhao, Maoliang Cheng, Wufeng Yuan
Lupus anticoagulants is related to both recurrent thrombosis and cancer. Thrombotic complications occur more frequently in patients with lung cancer. The aim of this study is to investigate the association of lupus anticoagulants with hypercoagulability and thrombotic complications, as well as prognostic significance of lupus anticoagulants for patients with lung cancer. The study comprised 205 patients with non-small cell lung cancer. Plasma normalized LAC ratio, D-dimer, fibrinogen, activities of antithrombin, and FVIII before treatment were analyzed by coagulation analyzer, and routine hematologic and biochemical parameters were also evaluated...
January 1, 2017: Technology in Cancer Research & Treatment
https://www.readbyqxmd.com/read/28588653/renal-cell-carcinoma-with-inferior-vena-cava-thrombus-extending-to-the-right-atrium-diagnosed-during-pregnancy
#4
REVIEW
Efe C Ghanney, Jaime A Cavallo, Matthew A Levin, Ramachandra Reddy, Jeffrey Bander, Maria Mella, Joanne Stone, Myron Schwartz, Kenneth Haines, Umesh Gidwani, Reza Mehrazin
Only one case of renal cell carcinoma (RCC) with inferior vena cava (IVC) tumor thrombus diagnosed and treated during pregnancy has been reported in the literature. In that report, the tumor thrombus extended to the infrahepatic IVC (level II tumor thrombus). In the present case, a 37-year-old woman with lupus anticoagulant antibodies was diagnosed with RCC and IVC tumor thrombus extending to the right atrium (level IV tumor thrombus) at 24 weeks of pregnancy. The fetus was safely delivered by cesarean section at 30 weeks of gestation...
December 2017: Therapeutic Advances in Urology
https://www.readbyqxmd.com/read/28574878/18f-fdg-pet-ct-imaging-of-popliteal-vasculitis-associated-with-polyarteritis-nodosa
#5
Romain Schollhammer, Paul Schwartz, Marie-Laure Jullie, Anne Pham-Ledard, Patrick Mercie, Philippe Fernandez, Jean-Baptiste Pinaquy
We report an F-FDG linear uptake of the right popliteal artery in a 25-year-old man who underwent a PET/CT scan for unexplained chronic asthenia associated with fever symptoms, arthralgia, and subcutaneous nodes, in favor of vasculitis. The patient had a history of saphenous vein thrombosis. Thrombophilia screening finds a blood lupus anticoagulant. A biopsy of subcutaneous nodes finds an anatomopathologic lesion of polyarteritis nodosa. F-FDG PET can be useful in initial staging of polyarteritis nodosa for an early diagnosis of vasculitis, and thus, early treatment can prevent arterial complications such as occlusion or aneurysm...
June 1, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28549386/newly-developed-dilute-russell-s-viper-venom-reagents-for-lupus-anticoagulant-detection-with-improved-specificity
#6
G W Moore, M Peyrafitte, C Dunois, J Amiral
Background Dilute Russell's viper venom time (dRVVT) is indispensible in lupus anticoagulant (LA) detection yet commercial reagents from different suppliers perform variably, no gold standard assays exist and therapeutic anticoagulation interference is problematic. Objective The objective of this study was to compare a new formulation dRVVT with two currently available dRVVTs. Materials and methods Life Diagnostics (LD) dRVVT and Stago PTT-LA were routinely used for lupus anticoagulant detection, plus Taipan snake venom time/ecarin time (TSVT/ET) for patients on warfarin or rivaroxaban...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28532221/antiphospholipid-antibodies-are-associated-with-positive-screening-for-common-mental-disorders-in-women-with-previous-pregnancy-loss-the-noha-psy-observational-study
#7
Jean-Christophe Gris, Fabienne Cyprien, Sylvie Bouvier, Eva Cochery-Nouvellon, Géraldine Lavigne-Lissalde, Erick Mercier, Jean-Pierre Balducchi
OBJECTIVES: Case reports describe neuropsychiatric manifestations associated with antiphospholipid antibodies (aPlAbs). In patients sharing the same symptoms fulfilling the antiphospholipid syndrome (APS) clinical criteria, the prevalence of common mental disorders has, however, never been studied. METHODS: We observed women with three consecutive abortions before the 10th week of gestation or one foetal loss at or beyond the 10th week. We compared the prevalence of common psychiatric disorders detected through screening using the Mini International Neuropsychiatric Interview, 10 years after inclusion, in women with APS (n = 506), women negative for aPlAbs but carrying the F5rs6025 or F2rs1799963 thrombogenic polymorphism (n = 269), and women with negative thrombophilia screening results as controls (n = 764)...
June 19, 2017: World Journal of Biological Psychiatry
https://www.readbyqxmd.com/read/28525532/the-effects-of-indirect-and-direct-acting-anticoagulants-on-lupus-anticoagulant-assays-a-large-retrospective-study-at-a-coagulation-reference-laboratory
#8
Jansen N Seheult, Michael P Meyer, Franklin A Bontempo, Irina Chibisov
Objectives: To investigate the effects of indirect- and direct-acting anticoagulants on the interpretation of lupus anticoagulant (LAC) assays. Methods: A retrospective database review was performed to identify all LAC panels from November 2012 to November 2015. The positivity rates for three LAC tests were compared among various anticoagulant medications. Results: This analysis included 7,721 LAC panels. Direct oral anticoagulants, warfarin, and unfractionated heparin (UFH) were associated with higher LAC positivity rates compared with patients not receiving documented anticoagulation (83% for argatroban, 58% for dabigatran, 72% for rivaroxaban, 53% for apixaban, 56% for warfarin, and 36% for UFH vs 29% for no anticoagulation, P < ...
June 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28516871/diagnosis-of-catastrophic-anti-phospholipid-syndrome-in-a-patient-tested-negative-for-conventional-tests
#9
Fabrizio Conti, Roberta Priori, Cristiano Alessandri, Roberta Misasi, Antonella Capozzi, Monica Pendolino, Simona Truglia, Silvia Frisenda, Maurizio Sorice, Guido Valesini
Catastrophic antiphospholipid syndrome (CAPS) is a severe variant of APS, characterised by clinical evidence of multiple organ involvement developing over a very short period of time, histopathological evidence of multiple small vessel occlusions and laboratory confirmation of the presence of aPL (lupus anticoagulant and/or anticardiolipin antibodies and/or anti-Beta2-glcyoprotein I antibodies). Here we report a case of a 39-year-old woman patient who developed a CAPS which was negative to the conventional aPL but positive for aPL in thin layer chromatography immunostaining and vimentin/cardiolipin antibodies by ELISA test...
May 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28502938/successful-treatment-of-catastrophic-antiphospholipid-antibody-syndrome-associated-with-malt-lymphoma-by-autologous-hematopoietic-stem-cell-transplantation
#10
Satoko Oka, Kazuo Ono, Masaharu Nohgawa
A 37-year-old woman with extranodal marginal-zone lymphoma was admitted with a fever, hemiplegia, and severe dyspnea after chemotherapy. Catastrophic antiphospholipid antibody syndrome (CAPS) was suspected based on the histopathological confirmation of small-pulmonary vessel occlusion, evidence of the involvement of three organs, and elevated lupus anticoagulant assay results in a short time span. The patient responded to the initial treatment. One month later, the CAPS and lymphoma relapsed, and the patient underwent autologous hematopoietic stem cell transplantation...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28501666/an-unexpectedly-high-rate-of-thrombophilia-disorders-in-patients-with-superficial-vein-thrombosis-of-the-lower-extremities
#11
Marcone Lima Sobreira, Silvia Regina Rogatto, Rodrigo Mattos Dos Santos, Izolete Thomazini Santos, Iracema Carvalho Ferrari, Winston Bonetti Yoshida
BACKGROUND: Superficial vein thrombosis is a common venous condition. Recent studies have shown that SVT is associated with high frequency of thromboembolic complications: from 22 to 37 % for deep venous thrombosis and up to 33% for pulmonary embolism. OBJECTIVES: To assess the prevalence of major hereditary and acquired thrombophilic factors in patients with SVT. PATIENTS AND METHODS: 66 patients presenting with primary ST underwent evaluation for thrombophilia: molecular testing for the factor V Leiden and factor II G20210A (prothrombin) mutations, protein C, protein S, antithrombin deficiency, presence of lupus anticoagulant, as well as anti-cardiolipin antibody titers...
May 10, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28474658/spontaneous-massive-hemopneumothorax-double-trouble-with-a-twist
#12
Milta Kuriakose, Arjun Khanna, Deepak Talwar
Spontaneous hemopneumothorax (SHP) is observed in 3%-7% cases of spontaneous pneumothorax where the tear of an adhesion can lead to bleeding with associated hemothorax. This condition has been reported in patients with hemophilia, sarcoidosis, congenital cystic adenomatoid malformation, systemic lupus erythematosus, etc., Here, we describe an unusual case of acute massive SHP in a 62-year-old male who underwent a percutaneous transluminal coronary angioplasty (PTCA) and presented with worsening dyspnea over the next 3 days...
May 2017: Lung India: Official Organ of Indian Chest Society
https://www.readbyqxmd.com/read/28471597/women-s-issues-in-antiphospholipid-syndrome
#13
Emmanouil Papadakis, Anastasia Banti, Anna Kioumi
Antiphospholipid syndrome (APS) is an autoimmune systemic disease characterized by vascular thrombosis (arterial or venous) and/or pregnancy complications associated with the occurrence of autoantibodies, specifically lupus anticoagulant, anticardiolipin antibodies, and/or anti-β2 glycoprotein-I antibodies confirmed at least twice over a 12 week period according to the 2006 Sydney criteria. Antiphospholipid antibodies are encountered  in the general population with a reported prevalence of 1% to 5%  However, APS is far more infrequent with a prevalence of 40-50/100,000 persons and an incidence of about 5 new patients/100,000 persons...
September 2016: Israel Medical Association Journal: IMAJ
https://www.readbyqxmd.com/read/28470650/the-clinical-relevance-of-noncriteria-antiphospholipid-antibodies
#14
Maria Laura Bertolaccini, Giovanni Sanna
While lupus anticoagulant (LA), anticardiolipin antibodies (aCL), anti-β2 glycoprotein I (anti-β2GPI) antibodies represent the best available and the most widely used tests in the investigation for antiphospholipid syndrome (APS), evidence gathered in recent years indicates that other antiphospholipid antibodies (aPL) specificities may also play a role in the syndrome. Several autoantibodies have been shown to be complexed with phospholipids other than cardiolipin, or to some domains of β2GPI, or else directed to other proteins of the coagulation cascade, and these have also been proposed to be of relevance to APS, and their diagnostic value and clinical utility are the focus of current research...
May 3, 2017: Seminars in Thrombosis and Hemostasis
https://www.readbyqxmd.com/read/28457197/management-of-cardiovascular-risk-in-systemic-lupus-erythematosus-a-systematic-review
#15
C Andrades, C Fuego, S Manrique-Arija, A Fernández-Nebro
Systemic lupus erythematosus is associated with accelerated atherosclerosis and increased risk of cardiovascular complications. The aim of this study was to review the effectiveness of interventions for primary and secondary prevention of cardiovascular events and mortality and to review the effectiveness of interventions for cardiovascular risk factor reduction in systemic lupus erythematosus patients. A systematic review was conducted. Electronic databases Medline and Embase (1961-2015) were searched. Nineteen articles met the inclusion criteria and were selected...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28447421/clinical-decision-support-for-hematology-laboratory-test-utilization
#16
REVIEW
R Jackups, J J Szymanski, S P Persaud
Clinical decision support (CDS) is the use of information and communication technologies to improve clinical decision making and patient care. CDS applications have been used in many aspects of health care, including medication ordering and diagnostic prediction algorithms. As economic and regulatory pressures place a strain on laboratory resources, the potential of CDS to improve utilization of laboratory testing has also begun to be realized. Hematology and coagulation laboratories stand to gain tremendously from the implementation of CDS interventions, given their mixture of high-volume, low-cost tests (eg CBC, PT, aPTT) and tests that carry a high potential of being misused or misinterpreted (eg lupus anticoagulant, erythrocyte sedimentation rate, heparin-induced thrombocytopenia testing)...
May 2017: International Journal of Laboratory Hematology
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
#17
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
#18
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
#19
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
#20
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
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