scholarly journals SAT0176 THERAPEUTIC APHERESIS DURING PREGNANCY IN RHEUMATIC DISEASES

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1029.2-1030
Author(s):  
A. Colpo ◽  
P. Marson ◽  
T. Tison ◽  
A. Zambon ◽  
A. La Rosa ◽  
...  

Background:Therapeutic apheresis (TA) represents a therapeutic option in pre-existing conditions or rheumatic diseases that occur during gestation. Although pregnancy is not a contraindication itself, due to the lack of evidence-based guidelines and the alleged risk of maternal and/or fetal adverse events, there is general resistance to its application during pregnancy.Objectives:In this observational study we aimed to evaluate the efficacy and safety of TA in high-risk pregnancies in patients with rheumatic diseases, followed over a decade in a tertiary Center.Methods:Between January 2005 and April 2019, 843 TA procedures were performed during 51 pregnancies in 43 patients: 745 plasma exchange sessions and 98 immunoabsorption sessions. TA was performed in 29 (57%) pregnancies of 21 (48.8%) patients with antiphospholipid antibody syndrome (APS), in 20 (39.2%) pregnancies of 20 (46.5%) patients with congenital heart block (CHB), in 1 (1.9%) pregnancy of 1 (2.3%) patient with systemic sclerosis (SSc) and 1 (1.9%) pregnancy of 1 (2.3%) patient affected by lupic nephritis (SLE).Results:During the period considered, apheresis sessions applied to pregnant women were 7.1% of the total (n = 13.251). The average age at the first treatment was 33 years (range 24-43). The mean management age at the first apheretic treatment was 21 weeks (range 4-32). Twelve (1.4%) apheresis sessions were complicated by adverse events, none required or prolonged hospitalization. There were 44 (86.3%) live births, 3 (5.9%) spontaneous abortions and 2 (3.9%) voluntary terminations of pregnancy, 2 (3.9%) lost to follow-up. The average gestational age at birth was 35 weeks (range 24-37) and cesarean section was performed in 41 (80.4%) cases. TA was added to conventional therapy in 24/29 (82.7%) patients with APS, to the detection of fetal cardiac activity, while in 5/26 (17.3%) it was introduced when the first signs of pregnancy complications such as mild preclampsia, HELLP and IUGR were detected. TA was started within 24 hours of atrioventricular block (AVB) detection; 10/20 (50%) mothers with CHB were diagnosed with 2nd degree AVB, 9/20 (45%) with 3rd degree AVB and one (5%) with sinus bradycardia and endocardial fibroelastosis. The patient with SSc was treated with TA twice a week from the 32nd SG until delivery, which occurred at the 36th SG, due to severe IUGR and oligohydramnios. The patient with SLE complicated by lupic nephritis was treated with TA twice a week, from the 26th SG until the birth, which took place at the 31st SG.Conclusion:Our data have shown that TA in pregnancy is well tolerated. Close collaboration between rheumatologist, obstetrician and specialist in TA is essential to ensure a successful outcome of high-risk pregnancies.Disclosure of Interests: :Anna Colpo: None declared, Piero Marson: None declared, Tiziana Tison: None declared, Alessandra Zambon: None declared, Annalisa La Rosa: None declared, Ermella Zanetti: None declared, Amelia Ruffatti: None declared, Giustina De Silvestro: None declared, Ariela Hoxha Speakers bureau: Celgene, UCB, Novartis, Sanofi, Werfen

2019 ◽  
Vol 46 (04) ◽  
pp. 435-445
Author(s):  
Marianna Politou ◽  
Vasiliki Mougiou ◽  
Maria Kollia ◽  
Rozeta Sokou ◽  
Georgios Kafalidis ◽  
...  

AbstractPrimary hemostasis, similar to other systems in the adjusting and transitioning neonate, undergoes developmental adaptations in the first days of life. Although platelets of neonates do not differ quantitatively compared with those of adults, they functionally present with major differences, thus supporting the theory of a “hypofunctional” phenotype that is counterbalanced by high hematocrit and more potent von Willebrand factor multimers. No clinical effect of bleeding tendency has hence been established so far for healthy term neonates. However, discrepancies in functionality have been noted, associated with gestational age, with more pronounced platelet hyporesponsiveness in preterm neonates. Multiple methods of in vitro platelet function evaluation such as PFA-100/200, platelet aggregometry, flow cytometry, and cone and platelet analyzer have been used for assessment of neonatal primary hemostasis. Several pregnancies are characterized as “high-risk” when risk factors preexist in maternal history or evolve during pregnancy. These pregnancies require specialized observation as they may have unpredictable outcome. High-risk pregnancies include clinical entities such as preeclampsia, pregnancy-induced smoking during pregnancy, gestational diabetes mellitus (GDM), autoimmune diseases, and other maternal hematological conditions. In some cases, like systemic lupus erythematosus, antiphospholipid antibody syndrome, and maternal immunologically based thrombocytopenia, neonatal thrombocytopenia is regarded as a prominent hemostasis defect, while in others, like pregnancy-induced hypertension and preeclampsia, both quantitative and qualitative disorders of neonatal platelets have been reported. In other pathologies, like GDM, neonatal primary hemostasis remains vastly unexplored, which raises the need for further investigation. The extent to which primary hemostasis is affected in neonates of high-risk pregnancies is the main objective of this narrative review.


2021 ◽  
Vol 23 (Supplement_G) ◽  
Author(s):  
Francesca Romana Prandi ◽  
Marialucia Milite ◽  
Roberto Celotto ◽  
Dalgisio Lecis ◽  
Massimo Marchei ◽  
...  

Abstract Antiphospholipid antibody syndrome (APS) is a systemic autoimmune disorder characterized by venous and arterial thromboembolic (TE) disease, and/or pregnancy morbidity, associated with persistent elevated antiphospholipid antibodies (lupus anticoagulant, LA, anticardiolipin, aCL, and anti-beta2-glycoprotein I, anti-β2GPI). A 51 year-old man, smoker, presented to our ER with chest pain. EKG showed Q waves and STE in the anterior leads, with increased troponin levels. A diagnosis of anterior STEMI was made and he was taken into our catheterization laboratory, where a high burden thrombotic plaque determining LM and ostial LAD subocclusion was treated with PPCI and a DES implantation; in addition, multiple failed PCI attempts were performed on a distal LAD occlusion, and an integrilin bolus was administered. The patient was admitted to our CICU and a DAPT with ASA and Ticagrelor was started. TTE showed LVEF 40%, apical akinesis, septal and anterior hypokinesis, and no significant valve disease. An aPTT abnormal value (100.5 s; reference range 25–38.5 s) was detected, not corrected by aPTT mixing study. Given suspected autoimmune prothrombotic state, normal Hb and PLT values and low bleeding risk, LMWH 100 IU/kg every 12 h was started, in addition to DAPT with switch from Ticagrelor to Clopidogrel. LA and aCL and anti-β2GPI IgG were positive, with negativity of others rheumatologic tests, confirming primary APS diagnosis. Repeat TTE showed EF 50%. The patient was discharged in ‘triple therapy’ (DAPT plus LMWH), and referred to the rheumatology clinic. LA, aCL, and anti-β2GPI tests 12 weeks later were positive, confirming a triple positive APS and satisfying revised Sapporo criteria. The rheumatologist switched therapy to Clopidogrel plus Warfarin. At 3 months follow-up, the patient, former smoker, was asymptomatic and TTE confirmed EF 50%, so he was recommended to continue with his medical therapy. APS is rarely associated with AMI (∼5.5%) and in only 2.8% cases AMI represents the onset of the disease. AMI pathogenesis in APS is considered to be acute thrombosis of coronary arteries, in contrast with atherosclerotic plaque rupture in typical AMI. Key discriminators to identify APS as potential underlying cause of AMI include young age, previous unprovoked thromboses, low platelets count (they are consumed in the thrombotic process), high aPTT value (LA may interfere with assembly of the prothrombinase complex on phospholipids), coronary artery thromboses in the setting of otherwise normal otherwise appearing coronary arteries. APS antibodies have also pro-inflammatory activity on vascular endothelial cells, leading to accelerated atherosclerosis. aGAPSS score (high risk ≥10) is useful for risk stratification of recurrent thrombosis and AMI in young patients with APS. The treatment of AMI in APS is therefore a clinical challenge. Strict management of additional CV risk factors is crucial. VKA (INR >3 with Warfarin, INR2–3 with Warfarin and ASA) should be provided for life, because of the very high risk of recurrent TE. DOACs are less effective and less safe than VKAs for TE prevention in APS. The role of coronary stents, considering higher rates of stent thrombosis after PCI in APS patients triple therapy concomitant risks, requires further studies. Due to lack of large, randomized, prospective studies, there is no clear experts consensus about optimal antithrombotic therapy in secondary prevention after arterial TE. APS patients with STEMI should undergo PCI, usually associated with thrombus aspiration, and in selected cases DES implantation in culprit lesion followed by triple antithrombotic therapy with short-term DAPT and long-term VKA.


2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
M Beringuilho ◽  
D Faria ◽  
A Freitas ◽  
F Bernardo ◽  
A Rego ◽  
...  

Abstract Introduction In the last decade there has been an intense discussion in which therapeutic option is most suitable for patients with TIA or cryptogenic AIS and a PFO. Recently two randomised studies (CLOSE and REDUCE) have been published tackling this question. Both have showed preference in lower recurrence rates in the group assigned for PFO closure. The eligibility criteria of these types of studies frequently are very restricted, becoming difficult to ascertain the best therapeutic option for a large number of patients. Goal Analyse a real world cohort of patients with TIA or cryptogenic AIS with PFO and identify the eligibility of these patients for the inclusion in the CLOSE and REDUCE trials. Methods We made a retrospective observational analysis of a cohort of patients discussed in a multidisciplinary meeting (Cardiology and Neurology departments) of our Hospital in which is made the decision of PFO closure vs conservative management between November 2017 and November 2018. We included all the patients with probable TIA or AIS and PFO. Demographic, clinical, image and therapeutic data was registered. The inclusion and exclusion criteria used in CLOSE and REDUCE trial were applied. Results 25 patients were analysed, 56.0% (n = 14) were male. Median age of 47 years. Median RoPE score 7. The commonest cardiovascular risk factors were hypertension (36.0%, n = 9) and smoking (28.0%, n = 7). Eight patients met criteria for cortical cryptogenic AIS, three had lacunar strokes, two had TIA with findings in cerebral imaging and twelve presented with TIA without findings in cerebral imaging. Transesophagic echocardiography was performed in 92.0% (n = 23) of patients, transthoracic echocardiography with bubble study was performed in two cases. At the time of data collection, six patients were waiting completion of the workup. PFO closure was performed in three cases. Closure was proposed in other two. When applying the eligibility criteria of CLOSE and REDUCE the main reasons for exclusion in the REDUCE trial were TIA without cerebral imaging (48.0%, n = 12), incomplete antiphospholipid antibody syndrome screening (36.0%, n = 9) and age greater than 59 years (28.0%, n = 7). The main reasons for exclusion in the CLOSE trial were TIA without cerebral imaging (48.0%, n = 12), age greater than 60 years (28%, n = 7) and failing to meet the designated echocardiography criteria (20.0%, n = 5). Conclusion 2 real world patients met eligibility criteria for the REDUCE trial and 3 for CLOSE. The main reason for exclusion in both REDUCE and CLOSE was TIA without cerebral imaging (48.0% n = 12). Older patients, and patients with a prothrombotic condition were also excluded. Multidisciplinary meetings are essential to ascertain the most beneficial therapeutic option for these patients. It is our believe that the management of risk factors should be similar in a TIA and a AIS and also that the presence of a prothrombotic condition should favor the closure of PFO.


Blood ◽  
2018 ◽  
Vol 132 (13) ◽  
pp. 1357-1358 ◽  
Author(s):  
Marissa Laureano ◽  
Mark A. Crowther

In this issue of Blood, Pengo et al present a randomized control trial comparing the use of rivaroxaban and warfarin in high-risk patients with antiphospholipid antibody syndrome (APS). The trial was stopped early because of an increased number of events in the rivaroxaban arm.1


VASA ◽  
2020 ◽  
Vol 49 (4) ◽  
pp. 333-337 ◽  
Author(s):  
Francisco Leonardo Galastri ◽  
Leonardo Guedes Moreira Valle ◽  
Breno Boueri Affonso ◽  
Marcela Juliano Silva ◽  
Rodrigo Gobbo Garcia ◽  
...  

Summary: COVID-19 is a recently identified illness that is associated with thromboembolic events. We report a case of pulmonary embolism in a patient with COVID-19, treated by catheter directed thrombectomy. A 57 year old patient presented to the emergency center with severe COVID-19 symptoms and developed massive pulmonary embolism. The patient was treated with catheter directed thrombolysis (CDT) and recovered completely. Coagulopathy associated with COVID-19 is present in all severe cases and is a dynamic process. We describe a case of massive/high risk pulmonary embolism, in a patient with COVID-19 receiving full anticoagulation, who was treated by percutaneous intervention. CDT can be an additional therapeutic option in patients with COVID-19 and pulmonary embolism that present with rapid clinical collapse.


1994 ◽  
Vol 71 (04) ◽  
pp. 424-427 ◽  
Author(s):  
Masahide Yamazaki ◽  
Hidesaku Asakura ◽  
Hiroshi Jokaji ◽  
Masanori Saito ◽  
Chika Uotani ◽  
...  

SummaryThe mechanisms underlying clinical abnormalities associated with the antiphospholipid antibody syndrome (APAS) have not been elucidated. We measured plasma levels of lipoprotein(a) [Lp(a)], the active form of plasminogen activator inhibitor (active PAI), thrombin-antithrombin III complex (TAT) and soluble thrombomodulin (TM), to investigate the relationship of these factors to thrombotic events in APAS. Mean plasma levels of Lp(a), TAT, active PAI and TM were all significantly higher in patients with aPL than in a control group of subjects. Plasma levels of Lp(a) and active PAI were significantly higher in patients with aPL and arterial thromboses than in patients with aPL but only venous thromboses. There was a significant correlation between plasma levels of Lp(a) and active PAI in patients with aPL. These findings suggest that patients with aPL are in hypercoagulable state. High levels of Lp(a) in plasma may impair the fibrinolytic system resulting in thromboses, especially in the arterial system.


1997 ◽  
Vol 77 (04) ◽  
pp. 624-628 ◽  
Author(s):  
Sabine Eichinger ◽  
Ingrid Pabinger ◽  
Andreas Stümpfien ◽  
Mirko Hirschl ◽  
Christine Bialonczyk ◽  
...  

SummaryThromboprophylaxis with oral anticoagulants up to six months is established in patients after a first venous thromboembolic event (VTE). The risk of recurrent VTE is still considerable thereafter, and it is uncertain whether some patients might benefit from extended anticoagulation. We performed a prospective, multicenter trial (4 thrombosis centers) and evaluated in 380 patients with a first or recurrent VTE (patients with a deficiency of antithrombin, protein C, protein S or plasminogen; cancer; or an antiphospholipid antibody syndrome were excluded) the risk of recurrence after discontinuation of secondary thromboprophylaxis with oral anticoagulants. It was the aim of the study to evaluate whether patients with factor V Leiden are at an increased risk of recurrent VTE. 112 (29.5%) patients were carriers of factor V Leiden (26.9% heterozygous, 2.6% homozygous). After a median observation time of 19.3 months the overall recurrence rate of VTE was 9.9%. Recurrent deep vein thrombosis and/or pulmonary embolism occurred in 26 of 268 patients without factor V Leiden (9.7%) and in 10 of 112 patients with factor V Leiden (8.9%). The probability of recurrent VTE two years after discontinuation of oral anticoagulants was 12.4% (95% Cl 7.8-17) in patients without factor V Leiden and was 10.6% (95% Cl 3.8-17.4) in carriers of the mutation. This difference was statistically not significant. Patients with factor V Leiden are not at a higher risk of recurrent VTE within two years after discontinuation of oral anticoagulants than patients without factor V Leiden. Balancing the risk of recurrent VTE and bleeding from oral. anticoagulants, patients with factor V Leiden are not likely to benefit from oral anticoagulant therapy extended beyond six months.


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