High-Risk Pregnancies and Their Impact on Neonatal Primary Hemostasis

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.

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


2021 ◽  
Vol 14 (1) ◽  
pp. e236592
Author(s):  
Ying Ling ◽  
Mary Jane Bell ◽  
Lisa Chodirker ◽  
Shirley Lake

A high functioning 74-year-old man with systemic lupus erythematosus presented to the emergency department with acute anxiety. He was found to have elevated cardiac enzymes and admitted to the cardiology service for investigation. In hospital, he developed an erythematous papular rash, and deteriorated to being somnolent and bedridden. He was found to have new multiterritory ischaemic strokes. It was eventually noted that he had persistent eosinophilia, present even on admission, which had been overlooked as the total leucocyte count was normal. Serology for antiphospholipid antibody syndrome (APS) was positive. He was diagnosed with hypereosinophilic syndrome (HES) secondary to new APS, and responded to high-dose steroids. This case highlights the importance of fully evaluating a leucocyte differential to make a diagnosis of HES. We discuss the definition, clinical manifestations, diagnostic approach and management of this important condition.


1998 ◽  
Vol 79 (02) ◽  
pp. 282-285 ◽  
Author(s):  
Josep Ordi-Ros ◽  
Francesc Monegal-Ferran ◽  
Nuria Martinez ◽  
Fina Cortes-Hernandez ◽  
Miquel Vilardell-Tarres ◽  
...  

SummaryObjective: To evaluate the usefulness of IgA antiphospholipid antibodies as markers of thrombosis and/or antiphospholipid antibody syndrome. Patients and Methods: A cross-sectional study design in a tertiary, university-based, autoimmune reference hospital. Seven-hundred ninety-five patients classified into five different groups – autoimmune diseases (255), deep vein thrombosis (153), transitory ischemic attacks (108), obstetric complications (196), infectious diseases (83) and controls (81) – were tested for IgA, IgG and IgM aPL, and lupus anticoagulant. Plasma and serum samples were drawn for detection of aPL using an internationally standardized ELISA method and LA was carried out using coagulometric assays. Results: True IgA aPL were found only in two patients with systemic lupus erythematosus; these patients were also positive to IgG aPL. Conclusion: The incidence of true positivity to IgA anticardiolipin antibodies is extremely low. Their determination was not helpful in diagnosing the antiphospholipid syndrome or in explaining thrombotic events or aPL related manifestations – fetal loss – in the groups studied.


2018 ◽  
Vol 2018 ◽  
pp. 1-4 ◽  
Author(s):  
Myat Han Soe ◽  
Krishna Adit Agarwal ◽  
Alueshima Akough-Weir

Catastrophic antiphospholipid syndrome (CAPS) is a rare but highly fatal clinical syndrome that occurs in up to 1% of patients with antiphospholipid syndrome (APS). The diagnosis of CAPS is often delayed because its presentation with multiple organ thromboses can be confused with other thrombotic microangiopathies and severe sepsis. We report a case of CAPS in a patient with APS and systemic lupus erythematosus (SLE) presenting with thrombotic storm precipitated by trauma, cytomegalovirus (CMV) infection, and noncompliance with anticoagulation therapy. Our case reflects the “two-hit hypothesis” of APS in which the presence of antiphospholipid antibodies (first hit) increases the thrombophilic risk, and thromboses take place in the presence of another thrombophilic condition such as CMV infection in our case. In this case review, we discuss the diagnostic challenges and management of CAPS. In clinical practice, we aim to stress the importance of thorough evaluation and management of precipitating events such as infections in addition to timely diagnosis and treatment of this catastrophic clinical entity.


2020 ◽  
Author(s):  
metin kaya gürgöze ◽  
Aslıhan Kara ◽  
Mehmet yusuf sarı ◽  
İlknur Çalık ◽  
Saadet Akarsu

Abstract Background: Although pyoderma gangrenosum (PG) -like lesions have been rarely described in adults with the antiphospholipid antibody syndrome (APS) and systemic lupus erythematosus (SLE), the occurrence of PG as a preceding manifestation of APS in children with SLE has not been reported until. We present a young girl with SLE and APS who developed progressive extstensive ulcerations that were consistent with PG.Case presentation: A 14-year-old girl with a 2-year history of SLE was admitted to our department, complaining painful crusted ulcerations on her legs. Skin biopsy was reported as PG. However, she did not respond to immunosuppressive therapy administered. When her skin biopsy findings is reassessed in keeping with the positive anticardiolipin antibody results, superficial small vessel microthrombosis was observed. Diagnosis of APS and PG developing secondary to SLE were made. It was resulted in marked clinical improvement with anticoagulation therapy in addition to immunosuppressives as is recommended in APS. Conclusions: Based in clinical, pathological and response to proposed treatment, we can state that PG -like lesions in children with SLE could be considered as a secondary form of APS.


Author(s):  
Alan J. Hakim ◽  
Gavin P.R. Clunie ◽  
Inam Haq

Introduction 344 Epidemiology and pathology 345 Clinical features of antiphospholipid syndrome 346 Treatment of antiphospholipid syndrome 348 Catastrophic antiphospholipid syndrome 350 The antiphospholipid syndrome (APS) was first described in the 1980s and comprises arterial and venous thrombosis with or without pregnancy morbidity in the presence of anticardiolipin (ACL) antibodies or the lupus anticoagulant (LAC). It can be primary, or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE) (...


2011 ◽  
Vol 2 (2) ◽  
pp. 102-104
Author(s):  
Joseph Theodore ◽  
P. Chitrambalam ◽  
K. Pradeep ◽  
S. Viswakumar

Antiphospholipid antibody syndrome (APLA) is a non-inflammatory autoimmune disease characterised by spontaneous abortion, thrombocytopenia and thrombosis (arterial and venous). Intracardiac thrombosis is a rare complication of APLA, but coronary sinus thrombosis in APLA has hitherto not been reported. We recently treated a young woman with secondary APLA and systemic lupus erythematosus in whom coronary sinus thrombosis was detected in association with recurrent pulmonary embolism. Key Words: intracardiac thrombosis; antiphospholipid antibody syndrome; systemic lupus erythematosus; coronary sinus thrombosis DOI: http://dx.doi.org/10.3126/ajms.v2i2.3885 Asian Journal of Medical Sciences 2 (2011) 102-104


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