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Antiphospholid Syndrome - Clinical
 

Antiphospholid Syndrome - Clinical

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    Antiphospholid Syndrome - Clinical Antiphospholid Syndrome - Clinical Presentation Transcript

    • Antiphospholipid Syndrome Ob/Gyn Presentation Joel E. Rodriguez RamosSunday, February 10, 13
    • Case • 26 year old woman attends the afternoon clinics for a routine examination of her current pregnancy. The patient is 11 weeks pregnant, G6 P1 A4, with some scant vaginal spotting on occasions. Upon further evaluation the patient states that her previous abortions were during the first trimester and spontaneous in nature. The patient also states no prior history of illness and denies coagulopathies. Upon follow-up patient presents laboratories with positive ANA and Anticardiolipin antibodies.Sunday, February 10, 13
    • Differential Diagnosis • Inherited Thrombophilia • Thrombotic Thrombocytopenic Purpura • Disseminated Intravascular Coagulation (DIC) • Systemic Lupus Erythematous • Anti-Phospholipid SyndromeSunday, February 10, 13
    • Epidemiology • Prevalence: 5.6% • Mean onset: 34 y/o • Affects Females 5:1 over Males • Associated with: • SLE = ~40% • Recurrent pregnancy loss = ~20% • Thrombosis = ~14% • RA = ~6%Sunday, February 10, 13
    • Etiology • No specific source has been identified • Elevated antiphospholipid antibodies bind to various phospholipid-binding plasma proteins (PBPP) forming venous, arterial, and microvascular thromboses and/or pregnancy associated morbidity.Sunday, February 10, 13
    • • Phospholipid-Binding Plasma Proteins include: • Apolipoprotein-H (aka: !2-GlycoProtein I) • Annexin A5 • Phospholipids on Platelets and Trophoblasts cells • Lupus Anticoagulant • The Complement system, with the association of resistance to Annexin A5, has been implicated in the pathogenesis of thrombosis and fetal loss.Sunday, February 10, 13
    • Pathophysiology • Anti-Annexin A5 antibodies exposes phospholipid- dependent coagulants to coagulation. • Anti-Cardiolipin Antibodies binds to Apolipoprotein-H prevents Protein C from deactivating Factor VIIIa. • Lupus Anticoagulant antibodies bind to pro-thrombin, increasing its cleavage to thrombin, thus accelerating the formation of fibrin.Sunday, February 10, 13
    • Risk Factors • Primary • HLA-DR7 • Secondary • SLE or RA • HLA-B8 • HLA-DR2 • HLA-DR3 • Ethnicity • African American, Hispanics and Native AmericanSunday, February 10, 13
    • Diagnostic Criteria • One Serological test • Must be positive twice at least 12 weeks apart • Lupus Anti-Coagulant • Anti-Cadiolipin antibodies via ELISA • ApoH (Anti-!2-Glycoprotein I) antibodies • ANA • ds-DNASunday, February 10, 13
    • Diagnostic Criteria • One Clinical test • Arteriovenous Ultrasound Doppler • MRI Venography • 3+ unexplained consecutive spontaneous abortions before the 10th week of gestation.Sunday, February 10, 13
    • Next Step in Management? • Verify prothrombin time (PT) • Use of amidolytic Factor X assay if a suitable PT assay is not found • Anticoagulation therapy with an INR target between 2 and 3 • Antenatal heparin and low dose aspirin throughout pregnancy for women no prior history of thrombosis. • Low dose aspirin for women with a history of pre-eclampsia or fetal growth restriction • Post-partum thromboprophylaxisSunday, February 10, 13
    • Management Flow Chart LMW Heparin Acute Fetal Monitoring Thrombosis Post-partum Warfarin Prophylactic Low-Dose Aspirin + LMW Heparin Obstretic APS Fetal Monitoring Post-partum Warfarin Prophylactic Low-Dose Aspirin + LMW Heparin APS + Hx Fetal Monitoring Thrombosis Post-partum Warfarin aPL Low-Dose AspirinSunday, February 10, 13
    • ACOG Guidelines • Women with APS and no thrombotic history should receive prophylactic low-dose aspirin, alone or along with heparin, during pregnancy and the postpartum period (6-8 weeks). • Women with APS and a previous history of thrombosis should receive full anticoagulation throughout pregnancy and the postpartum period (6-8 weeks). • Frequent prenatal exams, serial ultrasonography, and antepartum testing after week 32 of gestation, or earlier in cases of fetal growth restriction, should be performed.Sunday, February 10, 13
    • Prognosis • Good prognosis of both the mother and fetus if the condition is diagnosed and managed properly. • Complications • Thrombus formation • Pulmonary emboli • Transient Ischemic Attack (TIA) • Placenta Abruptio • Fetal growth restriction • Abortion or still birth • Pre-eclampsia • Patients should have multidisciplinary follow-up monitoring by an gynecologist, hematologist and rheumatologist.Sunday, February 10, 13
    • Current Research • New oral anticoagulants • Direct thrombin inhibitors • Oral Anti-Xa inhibitors (dabigatran, rivaroxaban) • Demonstrated to be safe and efficacious primary and secondary thromboprophylactic agents in limited studies. • Hydroxychloroquine • Widely used in the treatment of patients with SLE and has been associated with a decreased risk of thrombotic events. • Shown to reverse the binding of antiphospholipid antibody-apoH complexes to phospholipid bilayers.Sunday, February 10, 13
    • Doubts?Sunday, February 10, 13
    • References • American Congress of Obstreticians and Gynecologists - http://www.acog.org • US Department of Health Guidelines - http://guidelines.gov • E-Medicine - http://www.emedicine.com • Medline Plus - http://www.nlm.nih.gov/medlineplus • Pub Med Health - http://www.ncbi.nlm.nih.govSunday, February 10, 13