A 32-year-old woman at 36 weeks and 2 days of pregnancy was admitted to the hospital on November 20, 2012. The patient had suffered from thrombocytopenia for 4 months and thrombosis of the lower extremities for 1 month. The patient did not have any history of cardiac disease, nephronia, urophthisis, hepatitis, or tuberculosis.
This patient was in her third pregnancy. The first pregnancy was a miscarriage at 18 weeks of gestation, 3 years previous. The second pregnancy resulted in fetal death at 24 weeks gestation, 2 years previous. Thrombocytopenia was found during both first and second pregnancies, platelet counts were ~90x109/L (standard range [SR], 100x109-300x109/L). Anticardiolipin antibody was positive (>10 RU/mL [SR, <10 RU/mL]) in her second pregnancy.
The patient was diagnosed with intrauterine gestation by color Doppler ultrasound at First Hospital, Bethune Faculty of Medical Sciences of Jilin University after 40 days menelipsis, in her third pregnancy. At 16 weeks of gestation, the titer of the ABO blood group antibody was 1:256. At 19 weeks of gestation, the platelet count was 88x109/L (SR, 100x109-300x109/L). At 29 weeks and 6 days of gestation, the patient complained that she felt a numb pain that became severe after movement, on her left leg. Examination of color Doppler ultrasound discovered intraluminal visible heterogeneous hypoechoic and hyperechoic dots, no clear boundary between diseased area and endothelium of blood vessel, no flow signal in the lumen of blood vessel, and an increase of vein diameter in superficial femoral vein, proximal part of deep femoral vein, popliteal vein, anterior tibial vein, posterior tibial vein, peroneal vein, and muscle vein. These indicated that thrombosis occurred in her deep vein of the left leg. Her physicians suggested that she needed thrombolytic and anticoagulant treatments. The patient and her relatives, however, refused treatments because they were concerned about possible side effects on the baby.
At 34 weeks and 2 days of gestation, the patient's platelet count was 52x109/L (SR, 100x109-300x109/L). Three days after, the blood pressure was 140/90 mmHg. At 36 weeks and 2 days of gestation, she was admitted to hospital. The fetal heart rate was 150 beats/minute. Fetal non-stress test was normal. The titer of ABO blood group antibody was 1:512. The platelet count was 53x109/L (SR, 100x109-300x109/L). The urinary protein was negative. The anticardiolipin antibody (IgG) was positive (52 RU/mL [SR, <10 RU/mL]). The anti-beta2-glycoprotein-I antibody (IgG) was also positive (150 RU/mL [SR, 0-20 RU/mL]). The lupus anticoagulant was negative.
After reviewing the patient's previous pregnancy history and records, we noted that the patient had two abortions, one missed abortion at 18 weeks, and a fetal death at 24 weeks. The fetal death at 24 weeks meets the clinical criteria of a single fetal death at greater than 10 weeks. At 16 weeks of her third pregnancy, she had venous thrombosis with lower platelet count, which meets another clinical criteria for APS. Clinical examinations demonstrated that her anticardiolipin antibody (52 RU/mL) and anti-beta2-glycoprotein-I antibody (150 RU/mL) were positive, which meets laboratory criteria for APS. Therefore, at 34 weeks of the third pregnancy, the patient was diagnosed as APS.
Unfortunately, the patient and her relatives still refused thrombolytic and anticoagulant treatments. Her physicians considered her baby and her situation and decided to stop her gestation.
At 36 weeks and 3 days of gestation, the patient delivered a healthy infant (an Apgar score of 10 at 1 minute, an Apgar score of 10 at 10 minutes, bodyweight 2,440 g, height 47 cm) by cesarean section. After delivery, the patient received 4,000 IU (equal to 40 mg) of low molecular weight heparin, subcutaneously, and was administered fluids. Three days after, the patient felt abdominal pain. Examinations revealed a platelet count at 80x109/L (SR, 100x109-300x1/L) and activated partial thromboplastin time (APTT) at 83.8 seconds (SR, 20-40 seconds). An abdominal computed tomography (CT) scan showed less clear boundary, multiple lamellar irregular low density areas at sizes of 0.7-2.4 cm, edge enhancement during arterial phase (A) and venous phase (V), and low density images during portal venous phase (P) with normal bile duct and gall bladder in the liver (Figure 1). These indicated that the patient had deep vein thrombosis in the liver and this caused infarcts in the liver. To improve the liver situation, the patient took 1.8 g of reduced glutathione once only and 5,000 IU of low molecular weight heparin, once a day.
On the next day, examinations uncovered a platelet count at 81x109/L (SR, 100x109-300x1/L), APTT at 79.1 seconds (SR, 23-40 seconds), fibrinogen degradation product at 11.2 mug/mL (SR, 0-5 mug/mL), fibrinogen at 5.29 g/L (SR, 2-4 g/L), aspartate aminotransferase at 151 U/L (SR, 5-40 U/L), and alanine aminotransferase of 173 U/L (SR, 5-40 U/L). On day 5 post-delivery, the patient started to have blurred awareness, right limb shaking, an inability to speak, and to twitch and bite her own tongue. Then, the patient gradually recovered her awareness, which appeared to be normal after 30 minutes. Examinations showed her blood pressure at 160/105 mmHg, heart rate at 110 beats/minute, saturation of blood oxygen at 96%, aspartate aminotransferase at 123 U/L (SR, 5-40 U/L), alanine aminotransferase at 215 U/L (SR, 5-40 U/L), white blood cell count at 7.30x109/L (SR, 4x109-10x109/L) and absolute neutrophil count 6.06x109/L (SR, 0.5x109-0.7x109/L). A CT scan of the head did not demonstrate any bleeding area or thrombotic lesion. A magnetic resonance image of the head discovered an intracerebral multiple abnormal signal, a short T1 signal in the longitudinal sinus, a diameter of the right pupil at 4.0 mm and the left at 3.5 mm. These results suggested that the patient may have had venous sinus thrombosis. The patient had reflection of light, positive bilateral pathologic reflex, and autonomic activities of limps. The patient continued to have anticoagulant treatment (5,000 IU of low molecular weight heparin, twice a day, hypodermic injection, and 1.8 g of reduced glutathione once). At the same time, the patient received dehydration treatment (50 g of mannitol, every 12 hours) to lower intracranial pressure and cefepime hydrochloride to prevent infection.
On day 6 post-delivery, the patient was suddenly unconscious and exhibited paroxysmal-decerebrate-rigidity at 4 am. The patient's breath became short. The heart rate increased to 130 beats/minute. A CT scan of the head showed low density areas at bilateral lobus occipitalis and lobus temporalis. These results indicated that right cerebral had infarction and hemorrhage, and hemorrhage had broken into ventricles. At 9 am, her heart rate was 152 beats/minute with blood pressure at 114/55 mmHg. Laboratory tests showed APTT at 81.8 seconds (SR, 23-40 seconds), prothrombin activity at 63% (SR, 80%-120%), fibrinogen at 4.67 g/L (SR, 2-4 g/L), and platelet count at 56x109/L (SR, 100x109-300x109/L). Diameter of her pupil was 4.5 mm in the right eye and 2.5 mm in the left eye. Direct and indirect light reflex disappeared on days 6 and 11. On day 11 post-delivery, the patient died, even with all possible rescue treatments.