Cerebral and vein thrombosis, transient protein S deficiency, and anticardiolipin antibodies

SummaryThe high prevalence of free protein S deficiency in human immunodeficiency virus (HlV)-infected patients is poorly understood. We studied 38 HIV seropositive patients. Free protein S antigen values assayed using the polyethylene-glycol precipitation technique (PEG-fS) were statistically lower in patients than in controls. These values using a specific monoclonal antibody-based ELISA (MoAb-fS) and the values of protein S activity (S-act) were not statistically different between patients and controls. C4b-binding protein values were not different from control values. In patients, PEG-fS values were lower than MoAb-fS values. Ten patients had a PEG-fS deficiency, 4 patients had a MoAb-fS deficiency and 8 had a S-act deficiency. Protein S activity and MoAb-fS were lower in clinical groups with poor prognosis and in patients with AIDS but PEG-fS was not. A trend for reduced S-act/MoAb-fS ratios was observed in patients. PEG-fS was negatively correlated with anticardiolipin antibody titers whereas MoAb-fS was not. The plasma of PEG-fS deficient HIV-patients contained high amounts of flow cytometry detectable microparticles which were depleted from plasma by PEG precipitation. The microparticles were partly CD42b and CD4 positive but CD8 negative. These microparticles were labelled by an anti free protein S monoclonal antibody. The observed differences between MoAb-fS and PEG-fS values were correlated with the amount of detectable plasma microparticles, just like the differences between MoAb-fS and S-act. Plasma microparticles correlated with anticardiolipin antibody titers.In summary, free protein S antigen in HIV infected patients is underestimated when the PEG precipitation technique is used due to the presence of elevated levels of microparticles that bind protein S. The activity of free protein S is also impaired by high levels of microparticles. The prevalence of free protein S deficiency in HIV positive patients is lower than previously published (4/38, -10%) and is correlated with poor prognosis. By implication, use of a PEG precipitation technique might give artefactually low free protein S antigen values in other patient groups if high numbers of microparticles are present. In HIV patients, high titers of anticardiolipin antibodies are associated with high concentrations of cell-derived plasma microparticles.

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Autoimmune Protein S Deficiency and Deep Vein Thrombosis after Chickenpox

Thrombosis and Haemostasis ◽

10.1055/s-0038-1650245 ◽

1996 ◽

Vol 75 (01) ◽

pp. 212-213 ◽

Cited By ~ 5

Author(s):

Flora Peyvandi ◽

Elena Faioni ◽

Gian Alessandro Moroni ◽

Alberto Rosti ◽

Luigi Leo ◽

...

Keyword(s):

Deep Vein Thrombosis ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Deep Vein

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Portal vein thrombosis caused by protein C and protein S deficiency associated with cytomegalovirus infection

The Journal of Pediatrics ◽

10.1016/s0022-3476(95)70355-1 ◽

1995 ◽

Vol 126 (4) ◽

pp. 586-588 ◽

Cited By ~ 46

Author(s):

Ravit Arav-Boger ◽

Shimon Reif ◽

Yoram Bujanover

Keyword(s):

Portal Vein ◽

Portal Vein Thrombosis ◽

Cytomegalovirus Infection ◽

Protein C ◽

Protein S ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency

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Malignant isolated cortical vein thrombosis with type II protein S deficiency: a case report

BMC Neurology ◽

10.1186/s12883-016-0597-0 ◽

2016 ◽

Vol 16 (1) ◽

Cited By ~ 1

Author(s):

Nobuhiko Arai ◽

Masanao Tabuse ◽

Akiyoshi Nakamura ◽

Hiromichi Miyazaki

Keyword(s):

Case Report ◽

Protein S ◽

Type Ii ◽

Protein S Deficiency ◽

Cortical Vein ◽

Vein Thrombosis ◽

Cortical Vein Thrombosis ◽

S Deficiency

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Chylopericardium due to Subclavian Vein Thrombosis in the Setting of Protein S Deficiency

Case Reports in Cardiology ◽

10.1155/2021/2232057 ◽

2021 ◽

Vol 2021 ◽

pp. 1-4

Author(s):

Ian Jackson ◽

Yaman Alali ◽

Abedel Rahman Anani ◽

Ali Nayfeh ◽

Arindam Sharma ◽

...

Keyword(s):

Pericardial Effusion ◽

Subclavian Vein ◽

Vena Cava ◽

Protein S ◽

Brachiocephalic Vein ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

Subclavian Vein Thrombosis ◽

S Deficiency ◽

Lymphatic Duct

Background. Chylopericardium is the accumulation of lymphatic fluid in the pericardial cavity. It can be idiopathic or secondary to trauma, cardiothoracic surgery, neoplasm, radiation, tuberculosis, lymphatic duct dysfunction, thrombosis, or other causes. We present a case of chylopericardium due to subclavian vein thrombosis in a patient with protein S deficiency. Clinical Case. A 48-year-old man with a history of protein S deficiency presented to the emergency department with shortness of breath and a productive cough. CT of the chest showed pulmonary emboli, moderate pericardial effusion, and a large thrombus of the superior vena cava, brachiocephalic vein, and subclavian veins. He developed echocardiographic evidence of cardiac tamponade so he underwent pericardiocentesis with drainage of milky-appearing fluid. Analysis of the fluid showed elevated triglycerides consistent with chylopericardium. The pericardial effusion reaccumulated, likely secondary to lymphatic duct obstruction due to his subclavian vein thrombus. Catheter-assisted thrombolysis was performed with resolution of the patient’s effusion and symptoms. Conclusion. Chylopericardium is a rare but important complication of subclavian vein thrombosis. Management is typically with surgical intervention, although our case represents successful treatment with catheter-assisted thrombolysis.

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Acute Renal Vein Thrombosis, Oral Contraceptives, and Protein S Deficiency: A Successful Catheter-Directed Thrombolysis

Annals of Vascular Surgery ◽

10.1016/j.avsg.2009.01.006 ◽

2009 ◽

Vol 23 (5) ◽

pp. 687.e1-687.e4 ◽

Cited By ~ 4

Author(s):

Hyung-Kee Kim ◽

Hyang Hee Choi ◽

Jong-Min Lee ◽

Seung Huh

Keyword(s):

Oral Contraceptives ◽

Renal Vein ◽

Protein S ◽

Renal Vein Thrombosis ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

Catheter Directed Thrombolysis ◽

S Deficiency

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HIT Type II without Thrombocytopenia in a 15-Year-Old Boy with Protein S Deficiency and Recurrent Deep Vein Thrombosis

30th Hemophilia Symposium Hamburg 1999 ◽

10.1007/978-3-642-18240-2_14 ◽

2001 ◽

pp. 114-116 ◽

Cited By ~ 1

Author(s):

T. Severin ◽

R. Ensenauer ◽

A. Wiedensohler ◽

K.-G. Fischer ◽

B. Zieger ◽

...

Keyword(s):

Deep Vein Thrombosis ◽

Protein S ◽

Type Ii ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Recurrent Deep Vein Thrombosis ◽

Deep Vein

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Different Risks of Thrombosis in Four Coagulation Defects Associated With Inherited Thrombophilia: A Study of 150 Families

Blood ◽

10.1182/blood.v92.7.2353 ◽

1998 ◽

Vol 92 (7) ◽

pp. 2353-2358 ◽

Cited By ~ 245

Author(s):

Ida Martinelli ◽

Pier Mannuccio Mannucci ◽

Valerio De Stefano ◽

Emanuela Taioli ◽

Valentina Rossi ◽

...

Keyword(s):

Protein C ◽

Factor V Leiden ◽

Protein S ◽

Factor V ◽

Superficial Vein ◽

Protein S Deficiency ◽

Vein Thrombosis ◽

S Deficiency ◽

Superficial Vein Thrombosis ◽

Coagulation Defects

AbstractDeficiency of the naturally occurring anticoagulant proteins, such as antithrombin, protein C and protein S, and activated protein C resistance due to the factor V Leiden gene mutation is associated with inherited thrombophilia. So far, no direct comparison of the thrombotic risk associated with these genetic defects is available. In this study, we wish to compare the lifetime probability of developing thrombosis, the type of thrombotic symptoms, and the role of circumstantial triggering factors in 723 first- and second-degree relatives of 150 index patients with different thrombophilic defects. We found higher risks for thrombosis for subjects with antithrombin (risk ratio 8.1, 95% confidence interval [CI], 3.4 to 19.6), protein C (7.3, 95% CI, 2.9 to 18.4) or protein S deficiency (8.5, 95% CI, 3.5 to 20.8), and factor V Leiden (2.2, 95% CI, 1.1 to 4.7) than for individuals with normal coagulation. The risk of thrombosis for subjects with factor V Leiden was lower than that for those with all three other coagulation defects (0.3, 95% CI, 0.1 to 1.6), even when arterial and superficial vein thromboses were excluded and the analysis was restricted to deep vein thrombosis (0.3, 95% CI, 0.2 to 0.5). No association between coagulation defects and arterial thrombosis was found. The most frequent venous thrombotic manifestation was deep vein thrombosis with or without pulmonary embolism (90% in antithrombin, 88% in protein C, 100% in protein S deficiency, and 57% in factor V Leiden), but a relatively mild manifestation such as superficial vein thrombosis was common in factor V Leiden (43%). There was a predisposing factor at the time of venous thromboembolism in approximately 50% of cases for each of the four defects. In conclusion, factor V Leiden is associated with a relatively small risk of thrombosis, lower than that for antithrombin, protein C, or protein S deficiency. In addition, individuals with factor V Leiden develop less severe thrombotic manifestations, such as superficial vein thrombosis.

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