Primary antiphospholipid antibody syndrome and cerebral ischemia: report on acute intervention in two cases and literature review with emphasis on therapeutic options

Thrombosis, thrombocytopenia, recurrent fetal loss and a variety of non-thrombotic neurological disorders have all been associated with antiphospholipid antibodies (aPL). Cerebral ischemia associated with aPL is the most common arterial thrombotic manifestation. Depression, cognitive dysfunction, depression and psychosis have all been associated with aPL. The presumed pathophysiologic mechanism underlying these manifestations is thought to be a result of cerebral ischemia in some, but not all cases. Seizures, chorea and transverse myelitis all appear to be associated with aPL. An interaction between aPL and central nervous system cellular elements rather than aPL-associated thrombosis seems to be a more plausible mechanism for these clinical manifestations. Migraine on the other hand, does not appear to be associated with aPL in either lupus or non-lupus populations. Neuroimaging studies show an increased frequency of brain abnormalities in patients with aPL, but none appear to be specific. The best treatment strategy for preventing neurological manifestations of aPL is not fully defined. For thrombotic manifestations, both antiplatelet and anticoagulant therapies have been suggested. In some patients, immunosuppressant therapy has been used. For non-thrombotic manifestations, some combination of immunosuppressant therapy and symptomatic treatment may be warranted.

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Variability of Clinical and Paraclinical Manifestations in Antiphospholipid Antibody Syndrome - Two Case Reports and Literature Review

Orthopedics and Rheumatology Open Access Journal ◽

10.19080/oroaj.2018.13.555855 ◽

2018 ◽

Vol 13 (1) ◽

Author(s):

Elena Laura Iliescu

Keyword(s):

Literature Review ◽

Case Reports ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome

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Neurological Aspects of Antiphospholipid Antibody Syndrome

Lupus ◽

10.1177/096120339600500503 ◽

1996 ◽

Vol 5 (5) ◽

pp. 347-353 ◽

Cited By ~ 52

Author(s):

SR Levine ◽

RL Brey

Keyword(s):

Cerebral Ischemia ◽

Sinus Thrombosis ◽

Brain Infarction ◽

Cerebral Venous Sinus Thrombosis ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Younger Age ◽

Ischemic Encephalopathy ◽

Large Case

Antiphospholipid antibodies (aPL) have been associated with a variety of neurological disorders, mostly linked to focal neuroparenchymal ischemia or infarction. Cerebral ischemia associated with the antiphospholipid syndrome (APS) occurs at a younger age than typical atherothrombotic cerebrovascular disease, is often recurrent, and high positive GPL values are usually linked to the presence of a lupus anticoagulant. When other features of the syndrome are not present and cerebral ischemia occurs only associated with anticardiolipin immunoreactivity, there appears to be no discerning features of these patients unless GPL > 40 for which recurrent thrombo-occlusive events appear to occur more frequently. Other neurological manifestations associated with aPL include cerebral venous sinus thrombosis, ocular ischemia, dementia, including ischemic encephalopathy, and chorea. The role of aPL in migrainous events is controversial and may not play a role in recent, large case-controlled studies. Most seizures in patients harboring aPL are associated with focal brain infarction.

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A perturbation of antithrombin-III and protein C coupling associates with an increase of anti-β2-glycoprotein I antibody in non-antiphospholipid antibody syndrome cerebral ischemia

Blood Coagulation & Fibrinolysis ◽

10.1097/00001721-200212000-00006 ◽

2002 ◽

Vol 13 (8) ◽

pp. 703-709 ◽

Cited By ~ 12

Author(s):

W H Chen ◽

Y F Kao ◽

M Y Lan ◽

Y Y Chang ◽

J S Liu

Keyword(s):

Cerebral Ischemia ◽

Protein C ◽

Antithrombin Iii ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Glycoprotein I

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Mesothelioma-associated antiphospholipid antibody syndrome presenting with cutaneous infarction and neuropathy

British Journal of Dermatology ◽

10.1046/j.1365-2133.1998.02289.x ◽

1998 ◽

Vol 138 (6) ◽

pp. 1092-1094 ◽

Cited By ~ 10

Author(s):

Tucker ◽

Coulson ◽

Salman ◽

Kendra ◽

Johnson

Keyword(s):

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome

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Plasma Levels of Lipoprotein(a) Are Elevated in Patients with the Antiphospholipid Antibody Syndrome

Thrombosis and Haemostasis ◽

10.1055/s-0038-1642454 ◽

1994 ◽

Vol 71 (04) ◽

pp. 424-427 ◽

Cited By ~ 42

Author(s):

Masahide Yamazaki ◽

Hidesaku Asakura ◽

Hiroshi Jokaji ◽

Masanori Saito ◽

Chika Uotani ◽

...

Keyword(s):

Plasma Levels ◽

Active Form ◽

Plasminogen Activator Inhibitor ◽

Arterial System ◽

Control Group ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Lipoprotein A ◽

Soluble Thrombomodulin ◽

Relationship Of

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.

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The Risk of Recurrent Venous Thromboembolism in Patients with and without Factor V Leiden

Thrombosis and Haemostasis ◽

10.1055/s-0038-1656023 ◽

1997 ◽

Vol 77 (04) ◽

pp. 624-628 ◽

Cited By ~ 139

Author(s):

Sabine Eichinger ◽

Ingrid Pabinger ◽

Andreas Stümpfien ◽

Mirko Hirschl ◽

Christine Bialonczyk ◽

...

Keyword(s):

Oral Anticoagulant Therapy ◽

Oral Anticoagulants ◽

Factor V Leiden ◽

Multicenter Trial ◽

Observation Time ◽

Antiphospholipid Antibody ◽

Antiphospholipid Antibody Syndrome ◽

Factor V ◽

Increased Risk ◽

Recurrent Vte

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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