Bleeding and re-thrombosis in primary antiphospholipid syndrome on oral anticoagulation

2005 ◽  
Vol 93 (04) ◽  
pp. 694-699 ◽  
Author(s):  
Antonio Ciampa ◽  
Maurizio Margaglione ◽  
Giovanna Scenna ◽  
Luigi Iannaccone ◽  
Vincenzo Brancaccio ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Oral Anticoagulation ◽  
Minor Bleeding ◽  
Primary Antiphospholipid Syndrome ◽  
Bleeding Rate ◽  
Inherited Thrombophilia ◽  
Major Bleeding Rate ◽  
Male Sex ◽  
Thrombosis Rate

SummaryThe aimof this studay was to compare bleeding and re-thrombosis in primary antiphospholipid syndrome (PAPS), mitral valve replacement (MVR) and inherited thrombophilia (IT) at different oral anticoagulation intensities. It entailed a prospective 8-year follow-up on 67 patients with PAPS, 89 with IT and 24 with MVR. Anticardiolipin (aCL) antibodies detected by Elisa and lupus anticoagulant by clotting assays. At INR 2–3 minor bleeding rate was higher in MVR (33.3) than PAPS (10.9) and IT (4.2)(p<0.0001). At INR 3–4 minor bleeding rate was higher in PAPS (142) than IT (33.3) and MVR (5.8)(p<0.0001). At either INR major bleeding rate were not significantly different across the three groups, but in PAPS major and minor bleeding rates were superior at INR 3–4 than INR 2–3 (p=0.02 and p<0.0001). Re-thrombosis rate was higher in PAPS than IT at INR 2–3 (4.0 vs 0.35) (p=0.01) and at INR 3–4 (10.5 vs. nil). The hazard ratio for re-thrombosis between PAPS and IT was 13 (95% IC 1.6–102.2, p=0.015). By regression analysis, baseline IgG aCL titre (>80 GPL) (p=0.001) and male sex (p=0.03) independently predicted re-thrombosis. In conclusion, in PAPS, high intensity oral anticoagulation was not superior to conventional intensity in preventing re-thrombosis but was offset by greater bleeding rates. Male sex and elevated baseline IgG aCL predicted re-thrombosis in PAPS that is 13-fold more re-thrombogenic than IT.

Five-Year Follow-Up by Transesophageal Echocardiographic Studies in Primary Antiphospholipid Syndrome

2005 ◽  
Vol 96 (4) ◽  
pp. 574-579 ◽  
Author(s):  
Maurizio Turiel ◽  
Piercarlo Sarzi-Puttini ◽  
Rossana Peretti ◽  
Sara Bonizzato ◽  
Sabrina Muzzupappa ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Primary Antiphospholipid Syndrome

Lymphocyte Disturbances in Primary Antiphospholipid Syndrome and Application to Venous Thromboembolism Follow-Up

2016 ◽  
Vol 53 (1) ◽  
pp. 14-27 ◽  
Author(s):  
Laurent Simonin ◽  
Elisabeth Pasquier ◽  
Christophe Leroyer ◽  
Divi Cornec ◽  
Julie Lemerle ◽  
...  
Keyword(s):  
Venous Thromboembolism ◽  
Antiphospholipid Syndrome ◽  
Primary Antiphospholipid Syndrome

Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study

Blood ◽  
2010 ◽  
Vol 116 (16) ◽  
pp. 3058-3063 ◽  
Author(s):  
Lucía Comellas-Kirkerup ◽  
Gabriela Hernández-Molina ◽  
Antonio R. Cabral
Keyword(s):  
Hemolytic Anemia ◽  
Antiphospholipid Syndrome ◽  
Autoimmune Hemolytic Anemia ◽  
Lupus Anticoagulant ◽  
Thrombotic Event ◽  
Classification Criteria ◽  
Response To Treatment ◽  
Primary Antiphospholipid Syndrome ◽  
Pregnancy Morbidity

Abstract The updated Sapporo classification criteria for antiphospholipid syndrome (APS) only include thrombosis or pregnancy morbidity as clinical criteria. To test this notion, we studied 55 patients (80% women) with hematologic manifestations. All fulfilled the laboratory criteria for primary APS. Thirty-five patients (64%) had thrombocytopenia, 14 (25%) had autoimmune hemolytic anemia, and 6 (11%) had both. Twenty-five patients (22 women, 88%) also fulfilled one clinical criterion for APS after a median follow-up of 13.2 years (range, 1.45-37 years), whereas the remaining 30 patients (22 women, 73%) have not had any thrombotic event nor pregnancy morbidity after a median follow-up of 5.4 years (range, 0.12-24 years). No patient developed systemic lupus erythematosus during follow-up. The hematologic manifestation was asynchronous with the APS onset in 84% of patients. The response to treatment was similar regardless of the APS status. Patients with definite APS were more frequently positive for the lupus anticoagulant (63%) than lupus anticoagulant-positive patients without APS (30%; odds ratio, 3.5; 95% confidence interval, 1.07-11.4; P < .02). Anticardiolipin or anti–β2-glycoprotein-I antibodies were highly prevalent among the study groups. Our study suggests that, depending upon their antiphospholipid profile, patients with hemocytopenias appear to comprise a peculiar subset of patients with APS; some develop thrombotic and/or obstetric APS whereas others continue with hematologic APS.

Oral anticoagulation cost in primary antiphospholipid syndrome

2018 ◽  
Vol 29 (1) ◽  
pp. 135-138 ◽  
Author(s):  
Antonio Ciampa ◽  
Chiara Salapete ◽  
Sabrina Vivolo ◽  
Paul R.J. Ames
Keyword(s):  
Antiphospholipid Syndrome ◽  
Oral Anticoagulation ◽  
Primary Antiphospholipid Syndrome

scholarly journals Hydroxychloroquine Therapy and Netosis Regulators Expression in Patients with Primary Antiphospholipid Syndrome

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5049-5049
Author(s):  
Bruna M Mazetto ◽  
Bidossessi Wilfried Hounkpe ◽  
Sabrina Saraiva ◽  
Joyce Maria Annichino Bizzacchi ◽  
Erich Vinicius De Paula ◽  
...  
Keyword(s):  
Gene Expression ◽  
Treatment Period ◽  
Antiphospholipid Syndrome ◽  
Primary Antiphospholipid Syndrome ◽  
Gene Expressions ◽  
The Mean ◽  
The Difference ◽  
Mean Differences ◽  
Relative Gene

Abstract Introduction: Primary antiphospholipid syndrome (PAPS) is an autoimmune pro-thrombotic condition that affects different vascular beds, with no detectable underlying diseases. Immunothrombosis is at the basis of thrombosis development in PAPS and neutrophil activation and generation of neutrophil extracellular traps (termed NETosis) have been described as part of the immunological process. NETosis involves the orchestrated participation of several proteins such as peptidyl arginine deaminase (PADI4), neutrophil elastase (ELANE) and myeloperoxidase (MPO). PADI4 mediates histone citrullination, so that inhibition of PADI4 could be considered a potential therapeutic strategy to prevent NETosis. Hydroxychloroquine (HCQ) is an anti-malarial drug prescribed for patients with autoimmune diseases as complementary treatment for prevention of immune activation and thrombosis. Whether HCQ treatment affects the NETosis process is not known. Objective: The aim of this study was to evaluate whether HCQ use is associated with the expression of NETosis regulators proteins PADI4, ELANE, and MPO in patients with PAPS. Methods: This is a cross-over study in which patients with PAPS were selected to receive HCQ at 400mg per day for 6 months and then discontinue the drug. The study had two periods of follow-up: 6 months of HCQ treatment and 6 months of no HCQ treatment. A wash-out period of 6 months between the two study periods was allowed. The mean gene expression and 95% confidence intervals (95% CI) of PADI4, ELANE, and MPO were calculated at baseline and at the end of the study periods. The within periods change in gene expression from baseline to the end of the treatment and the difference in gene expression between periods at the end of the follow-up were determined. Results: The study comprised 20 patients. Mean age was 45 years, 70% were women. In HCQ treatment period, mean relative gene expressions of PADI4, ELANE, and MPO were respectively: 1.0 (SD=0.6), 0.5 (SD=0.7) and 0.6 (SD=0.7) at baseline and 0.8 (SD=0.3), 0.9 (SD=1.5) and 0.6 (SD=0.9) at the end of the period. The mean changes in PADI4, ELANE, and MPO relative gene expressions during HCQ treatment period were: -0.1 (95%CI:-0.4;0.1), 0.4 (95%CI: -0.5;1.4), 0 (95%CI: -0.6;0.5), respectively. In the period when HCQ was not used, mean relative gene expressions of PADI4, ELANE, and MPO were respectively: 1.2 (SD=0.6), 0.6 (SD=1.4) and 0.8 (SD=1.3) at baseline and 0.8 (SD=0.4), 0.4 (SD=0.9) and 0.6 (SD=0.6) at the end of the period. The mean changes in PADI4, ELANE, and MPO relative gene expressions during period of no treatment were: -0.4 (95%CI:-0.6;-0.2), -0.2 (95%CI:-0.5;0.1) and -0.2 (95%CI -0.5;0), respectively. In comparison with no treatment period, treatment with HCQ had no effect on the relative gene expressions of PADI4, ELANE, and MPO. The mean differences in PADI4 ELANE, and MPO relative gene expression between HCQ treatment and no treatment at the end of the periods were 0.1 (95%CI:-0.1; 0.3), 0.5 (95%CI:-0.2; 1.3) and 0.1 (95%CI:-0.3; 0.5), respectively. Conclusion: The results suggest that HCQ treatment has no effect on NETosis process, as measured by the gene expression of NETosis regulators proteins, in patients with PAPS. Disclosures De Paula: Hematology and Transfusion Medicine Center, University of Campinas: Employment.

scholarly journals Primary antiphospholipid syndrome progressing to systemic lupus erythematosus: a case report

2013 ◽  
pp. 116-121
Author(s):  
Rocco Manganelli ◽  
Salvatore Iannaccone ◽  
Walter De Simone
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Primary Antiphospholipid Syndrome ◽  
Systemic Lupus ◽  
Anionic Phospholipids ◽  
Serological Studies ◽  
Homogeneous Pattern

Introduction: Primary antiphospholipid syndrome (APS) is a thrombophilic disease that should be suspected in the presence of thrombotic events associated with hematologic abnormalities such as thrombocytopenia and prolongation of the activated partial thromboplastin time. The diagnosis must be confirmed by the demonstration of autoantibodies directed against anionic phospholipids and/or phospholipid-binding proteins. The disease can cause arterial thrombosis in any vascular district, including those of the kidney and central nervous system. Case report: In 2006 a 29-year-old male presented with kidney and brain involvement that was attributed to primary APS. The clinical diagnosis was confirmed by the results of a renal biopsy, which excluded the presence of systemic lupus erythematosus (SLE). The patient remained stable through 32 months of follow-up and then developed a malar rash with deteriorating renal function, decreasing platelet count, and reduced complement levels. Serological studies revealed positivity for ANA (homogeneous pattern), dsDNA, ACA, and beta-2-glycoprotein-1 antibodies. The diagnosis was revised to APS secondary to SLE. Conclusions: A diagnosis of primary APS should not be considered permanent: progression to SLE can occur, in some cases years after the original diagnosis. This case highlights the importance of ongoing follow-up of patients diagnosed with primary APS to detect changes that herald the emergence of SLE.

Antibody profile and clinical course in primary antiphospholipid syndrome with pregnancy morbidity

2006 ◽  
Vol 96 (09) ◽  
pp. 337-341 ◽  
Author(s):  
Amelia Ruffatti ◽  
Marta Tonello ◽  
Teresa Del Ross ◽  
Anna Cavazzana ◽  
Chiara Grava ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Pregnancy Loss ◽  
Late Pregnancy ◽  
Primary Antiphospholipid Syndrome ◽  
Thromboembolic Events ◽  
Pregnancy Morbidity ◽  
Glycoprotein I ◽  
History Of ◽  
Classification Category

SummaryIn women diagnosed as having category I primary obstetric antiphospholipid syndrome, clinical characteristics and the risk of subsequent thromboembolic events and further unsuccessful pregnancy has not been clearly documented. Women with unexplained obstetric complications and no definite autoimmune systemic diseases were tested for lupus anticoagulant (LA), IgG/ IgM anticardiolipin (aCL) and IgG/IgM anti-human β2-Glycoprotein I (aβ2GPI) antibodies and diagnosed as having primary antiphospholipid syndrome (APS) in classification category I on the basis of more than one laboratory criteria present in any combination. Characteristics at the time of diagnosis and risk factors for subsequent clinical events during a mean follow-up of 6.3 years were evaluated. Fifty-three of 600 women studied were found to fulfil obstetric criteria and had more than one positive laboratory test at the time of diagnosis. All the women were a CL and aβ2GPI positive, and 16 were also LA positive. This latter group (triple positivity) had distinct features and had more frequently experienced previous thromboembolism (OR= 122.5, 95% CI 16–957, p<0.001).They also had an increased rate of late pregnancy loss (OR=16.2, 95%CI 0.9–292, p=0.01), and a higher IgG aβ2GPI titer at diagnosis (median, 25th and 75th percentile were 118, 37–962, vs. 23, 18–32, respectively, p<0.0001). During follow-up, the rate of thromboembolic events was significantly higher in the group of women with triple positivity and/ or previous thromboembolism (OR=57.5, 95% CI 2.7–1160, p=0.0004) which were the only independent predictors of TE in the multivariate model. Recurrent pregnancy loss took place in seven out of 47 women who had a new pregnancy. Triple positivity and/or previous thromboembolism were again the only independent markers (OR=34.4, 95% CI 3.5–335.1, p=0.003) of an unsuccessful new pregnancy. In conclusion, in primary APS with pregnancy morbidity in classification category I, quite different groups of patients may be identified on the basis of laboratory tests. Triple positivity and/or a history of thromboembolism predict new TE events and new unsuccessful pregnancies.

284Outcomes after left atrial appendage occlusion with AMPLATZER Amulet and WATCHMAN device: Results from the ORIGINAL registry (saxOnian RegIstry analyzinG and followINg left atrial Appendage cLosure)

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Kretzler ◽  
C Wunderlich ◽  
M Christoph ◽  
A Langbein ◽  
S G Spitzer ◽  
...  
Keyword(s):  
Atrial Fibrillation ◽  
High Risk ◽  
Oral Anticoagulation ◽  
Left Atrial ◽  
Left Atrial Appendage ◽  
Atrial Appendage ◽  
Minor Bleeding ◽  
Left Atrial Appendage Closure ◽  
Left Atrial Appendage Occlusion

Abstract Aims Left atrial appendage occlusion (LAAO) is a therapeutic option for patients with non-valvular atrial fibrillation (NVAF) and high risk of bleeding. This study reports outcomes of patients enrolled in the prospective multicentre, investigator initiated real life registry in the Free State of Saxony (saxOnian RegIstry analyzinG and followINg left atrial Appendage cLosure). Methods and results Data of all consecutive 521 patients (64.7% adult males, 35.3% adult females, mean age: 75.1 (SD 7.9) years with non-valvular atrial fibrillation undergoing interventional left atrial appendage occlusion procedure in the ORIGINAL prospective registry were analysed. The CHA2DS2-VASc and HAS-BLED scores were 4.3 (SD 1.5) and 3.7 (SD 1.1), respectively. 78.9% of the patients had a history of bleeding. 89.3% of the patients were considered as non-eligible for long term oral anticoagulation. A left atrial appendage occlusion device was successfully implanted in 97.5% of cases. A mean follow-up of 463 days could be reached in 386 patients. Among these, the distribution of the follow-up length reached was as follows: 1 year 205, 2 years 118, 3 years 65 and 4 years 17 patients. In the follow-up the annual frequency of stroke was very low (0.4%), which resulted in the 4.98% absolute risk reduction in the amount of thromboembolic strokes, which would have been expected according to the calculated CHA2DS2-VASc score in the hypothetic group not receiving any anticoagulant therapy. The occurrence of major and minor bleeding in the follow-up was 1.55% and 3.37% respectively. Conclusions In this prospective multicentre study we included the patients who are at high risk of stroke and bleeding. The annual ischemic stroke rate was 0.4%, the LAA could be sealed in 97.5%. Six months after the LAA closure only 3.63% of all patients were further on treated using oral anticoagulation. Considering the stroke reduction rates, the implantation of an LAAO device proves to be effective and safe in the clinical routine in the studied population. Acknowledgement/Funding None

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