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and pregnancy
Lech Dudarewicz, MD, Lucjusz Jakubowski, MD, PhD.
Synonyms
Definition
Diagnosis
The occurrence of one clinical criterion with one biological criterion defines the
syndrome.
Clinical criteria
Fetal loss: the strict criterion should be 3 or more spontaneous abortions with no
more than one live birth or unexplained second or third trimester fetal death.
Thrombosis: unexplained venous or arterial thrombosis including transient ischemic
attacks or amaurosis fugax.
Autoimmune disorder: thrombocytopenia of less than 100,000 cells/mm3, after
exclusion of other causes of thrombocytopenia, unexplained positive result from the
Coombs test, hemolytic anemia with reticulocytosis, after exclusion of other causes,
leukopenia of less than 4000 cells/mm3, lymphopenia of less than 1500 cells/mm3.
Neurologic disorder: chorea/chorea gravidarum. Criteria include seizures in the
absence of other causes or psychosis in the absence of other causes.
Cutaneous manifestations: peripheral ischaemia including cyanosis, ulcers, after
exclusion of other causes, discoid rash, photosensitivity.
Arthritis, serositis, in the absence of other causes.
Renal disorder: proteinuria of 0.5 g/d or the presence of cellular casts, without
another cause, is a criterion for APS.
Biological criteria
Biological criterion is each of the following:
Prevalence
Etiology
Pathogenesis
damage to the endothelial cells, shifting the balance between the production
of prostaglandin E2 and thromboxane
reactivity with platelet phospholipids, favoring platelet aggregation
interference with complement activation
Pregnancy complications
Associated anomalies
Very interesting is the hypothesis of Carolyn B Coulam, linking the presence of aPL
antibodies to congenital anomalies resulting from the vascular accidents attributable
to thrombus formation. The spectrum of these defects comprises oromandibular
hypogenesis and kidney defects. There are however too few observations to draw
firm conclusions.
Differential diagnosis
Prognosis
Good, if fractionated heparin and aspirin treatment is started before the pregnancy.
Of note is the fact, that untreated women with the diagnosis of antiphospholipid
syndrome have approximately 90% risk of fetal loss.
Recurrence risk
Management
Warning
Case report
A 26-year-old G3P3 woman had a past medical history significant for emergency
cesarean delivery in the 27th week of her second pregnancy, complicated by
imminent intrauterine asphyxia caused by placental insufficiency. The child died
soon after delivery due to hypoxia and prematurity. She was subsequently found
positive for anticardiolipin antibodies.
From the beginning of her third pregnancy she was placed on fractionated heparin
(Clexane). Until 19 weeks, the course of the pregnancy was uneventful. In the 19th
week the size of the fetus was discrepant with the menstrual age and with the
ultrasound dating from the first trimester. The head was two weeks smaller, than
expected, and the abdominal circumference, femur length and humerus length were
almost four weeks smaller than expected. The amniotic fluid volume was reduced
(AFI=6.5 cm). There was cardiomegaly and some thickening of the myocardium of
both ventricles of the heart. Echogenic intracardiac focus was present in the left
ventricle. The anatomy of the heart was otherwise normal: normal outflow tracts and
three-vessel view. There was absent diastolic flow in the umbilical arteries and
pulsatility was present in the umbilical vein. There was reversed A wave in the
ductus venosus. The placenta was thickened, approximately up to 3.7 cm and of
markedly inhomogeneous echogenicity. The bowel of the baby was admittedly no
more echogenic, than the iliac bones, but subjectively judged as of increased
echogenicity, even at low ultrasound frequency.
The patient was negative for TORCH infections. The fetal karyotype was 46, XY.
At 26 weeks the fetus died in utero. After birth no structural anomalies were noted.
The baby weighted 230 grams, which is below 3rd centile for 26 weeks.
The diagnosis of APS requires co-existence of clinical and laboratory findings. As far
as the reproductive process is concerned, in most patients either there is a fetal loss
attributable to abnormal placental function, or there is a normal course of pregnancy.
Our patient is distinctive, because of the fact of discovering very early symptoms of
placental failure, in spite of anticoagulant therapy.
Besides fetal loss, APS patients are at risk of venous thrombosis, even up to the
catastrophic embolus. One of the safety measures is color flow Doppler examination
of the IVC which is safe and inexpensive. The signs and symptoms of thrombosis
particularly in puerperal APS patients have very low reliability. In the APS patients a
high index of suspicion for venous thrombosis should be maintained.
References
- Aoki K, Matsuura E, Sasa H, Yagami Y, Dudkiewicz AB, Gleicher N. beta 2-
Glycoprotein I-dependent and -independent anticardiolipin antibodies in healthy
pregnant women. Hum Reprod. 9(10):1849-51; 1994
- Coulam CB. Hypothesis: antiphospholipid antibodies associated with congenital
anomalies? Early Pregnancy. 3(2):109-12; 1997