Anesthetic management in a case of antiphospholipid antibody syndrome.

Antiphospholipid antibody (APLA) syndrome is one of the most common thrombocytophilias but, unfortunately, goes unrecognized most often. It is an auto-immune disorder in which thrombotic events and a recurrent fetal loss occur in the presence of antibodies to phospholipids. It is the most common acquired hyper-coagulable state. There is a limited literature on peroperative management of patients with this syndrome. We report a case of APLA syndrome in a parturient due to its rarity and complexity.

INTRODUCTION

APLA syndrome is an autoimmune hypercoagulable state caused by antiphospholipid antibodies. It is characterized by thrombotic episodes in arteries, veins and pregnancy related complications like still birth, preterm delivery, miscarriage and severe preeclampsia. We report a case of APLA syndrome due to its rarity.

CASE REPORT

A 22-year-old woman with a history of two previous abortions and positive serology (presence of lupus anticoagulant and increase in antiphospholipid antibodies [APLAs] – 18.79 U/ml with a laboratory reference value of 12 U/ml) for APLAs with 9 months gestation came to our hospital for safe confinement. She was diagnosed as primary APLA syndrome in view of bad obstetric history and positive lupus anticoagulant, even though, there was no history suggestive of thromboembolic episodes. She had no history of hypothyroidism or other auto-immune disorders.

She was started on aspirin 75 mg OD and injection nadroparin 0.3 ml (9500 IU/ml) subcutaneously to improve fetal outcome. In view of bad obstetric history, an elective caesarean section was planned. Aspirin was stopped 3 days before surgery, and low molecular weight heparin was stopped on the day of surgery. Preoperative investigations revealed a normal activated partial thromboplastin time, prothrombin time, international normalized ratio.

Patient was premedicated with injection ranitidine 50 mg and injection perinorm 5 mg 30 min before the surgery. Spinal anesthesia given in L3–L4 inter-spinal space with 25G needle after preloading with 500 ml crystalloids and a sensory block up to sixth thoracic vertebral level was attained. She delivered a single live male child with APGAR score 9. 15 units oxytocin and 250 µg prostagladin F2α were administered to facilitate uterine contraction. The further perioperative course was uneventful. She was restarted on nadroparin for 6 weeks and asprin.

DISCUSSION

Antiphospholipid antibody syndrome is an auto-immune disorder with variable symptomatology ranging from recurrent thromboembolic events, recurrent abortions, valvular lesions, thrombocytopenia and hemolytic anemia.[12] The incidence is more in woman's than males (female: male 4.5:1). The diagnosis of the antiphospholipid syndrome is based on the occurrence of clinical features and positive serology for APLAs. Lupus anticoagulant, anticardiolipin and anti β2 glycoprotein are the most common antibodies associated with APLA. Clinical presentation of the syndrome occurs in only 2% of the population though the APLAs can be present in about 5% of the population.[3] 40–50% with lupus also have APLA syndrome.

It can be primary in the absence of any underlying illness or secondary when associated with other auto-immune disorders.[2] Despite their name, lupus anticoagulant antibodies are associated with thromboembolic events rather than clinical bleeding episodes. Although the pathogenesis of this syndrome is poorly understood, many mechanisms have been described, one being binding of APLAs to endothelial cells, which stimulates an up-regulation of the adhesion molecules and an increase leukocyte adhesion leading to a prothrombic state.[45] Most recently a catastrophic antiphospholipid variant with a mortality rate of approximately 50% has been described.[6] In the pregnant uterus, the fetoplacental endothelium is mainly targeted thus interfering with the implantation of the embryo causing recurrent abortion.

In patients with a history of miscarriage, future outcomes of the pregnancy are improved when a combination of aspirin and heparin are used.[67] This therapy is withheld at the time of surgery to decrease blood loss and started immediately after delivery and continued for 6 weeks postpartum.

Antiphospholipid antibody can be precipitated peroperatively (thrombotic storm) by surgical intervention, infection or a change in anticoagulation therapy.[6] Intraoperatively anti-embolic stockings, intermittent venous compression devices and adequate hydration can decrease the incidence of such complications. The optimal analgesic administration is imperative to facilitate early mobilization, which in turn decreases the incidence of thrombotic events postoperatively. Vigilant monitoring for both bleeding and thromboembolic episodes is required during the perioperative period.

CONCLUSION

A successful outcome in a patient with APLA syndrome requires a multidisciplinary approach to prevent both thrombotic and hemorrhagic complications. However, routine screening of pregnant women is not necessary because of it is low incidence.