Updated: 6/18/2018

Antiphospholipid Syndrome

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Snapshot
  • A 30-year-old woman presents to the physician's office for evaluation of recurrent fetal losses. She reports having had 3 past spontaneous abortions at <10 weeks of gestational age. She also reports having a history of deep venous thromboses. She denies ever being diagnosed with systemic lupus erythematosus but has been diagnosed with thyroid disease. Physical exam is remarkable for livedo reticularis on her thighs. Further blood testing reveals positive lupus anticoagulant and anticardiolipin antibody.
Introduction
  • Clinical definition
    • autoimmune disorder that is often associated with systemic lupus erythematosus (SLE), characterized by thromboses and recurrent spontaneous abortions
  • Epidemiology
    • incidence
      • increases with age
    • demographics
      • female > male
      • affects adults
    • risk factors
      • SLE
      • drugs
        • procainamide
        • isoniazid
        • hydralazine
        • oral contraceptives
        • malignancy
  • Pathogenesis
    • autoantibodies (lupus anticoagulant, anticardiolipin, and anti-β2 glycoprotein antibodies) react against platelet membranes or prothrombin-platelet membrane complex, activating endothelial cells and platelets 
      • which further activates complement-mediated thrombosis
  • Associated conditions
    • SLE
    • other autoimmune diseases
    • preeclampsia or eclampsia
  • Prognosis
    • risk of recurrence is high
Presentation
  • Symptoms
    • recurrent fetal loss
    • symptoms related to thrombosis site
      • dyspnea
      • abdominal discomfort
      • neurologic symptoms
      • chest pain
      • skin rash
  • Physical exam
    • cutaneous findings include livedo reticularis
      • reticular (lacy) or mottled violaceous rash
Studies
  • Labs
    • lupus anticoagulant
      • prolonged dilute Russell viper venom time (DRVVT) test
      • prolonged partial thromboplastin time (PTT)
    • anti-β2 glycoprotein (IgG and IgM)
    • anticardiolipin (IgG and IgM)
    • coagulation studies
      • normal or slightly prolonged prothrombin time
      • normal bleeding time
      • normal platelet count
    • false positives due to antibodies
      • + VDRL/RPR due to anticardiolipin antibodies
      • prolonged PTT due to lupus anticoagulant
        • not corrected by adding normal platelet-free plasma
  • Making the diagnosis
    • based on clinical presentation and laboratory studies
    • diagnostic criteria includes ≥ 1 clinical and ≥ 1 laboratory criteria
      • clinical
        • thrombosis
        • spontaneous abortion at ≥ 10 weeks gestational age
        • premature birth at < 35 weeks gestational age due to eclampsia, pre-eclampsia, or placental insufficiency
        • ≥ 3 spontaneous abortions before < 10 weeks gestational age
      • laboratory
        • lupus anticoagulant
        • anticardiolipin antibody
        • anti-β2 glycoprotein
Differential
  • Factor V Leiden mutation
    • distinguishing factor
      • absence of antibodies
  • Antithrombin deficiency
    • distinguishing factor
      • normal PTT
Treatment
  • Medical
    • warfarin
      • indications
        • for non-pregnant patients
        • prevention of thrombosis
        • target INR 2-3
    • aspirin plus unfractionated heparin
      • indications
        • for pregnant patients
        • prevention of pregnancy loss
Complications
  • Fetal loss
  • Stroke

R

 

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Questions (1)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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