Alpha II Antiplasmin Deficiency Complicating Pregnancy: A Case Report

Background. Alpha II antiplasmin is a protein involved in the inhibition of fibrinolysis. A deficiency in this protein leads to increased hemorrhage. It is inherited in an autosomal recessive fashion. Case. 30-year-old Gravida 1, Para 0, presented for prenatal care with her first and subsequently her second pregnancy. Her medical history was significant for a known deficiency in alpha II antiplasmin. Her first and second pregnancies were complicated by nonobstetrical hemorrhage requiring transfusions and severe preeclampsia requiring preterm deliveries. Conclusion. Alpha II antiplasmin deficiency resulted in multiple episodes of nonobstetrical hemorrhages requiring transfusion and ultimately preterm deliveries due to severe preeclampsia. Both infants and mother had a good outcome. The presence of this disorder may require a multidisciplinary team approach involving obstetricians, pediatricians, and hematologists. Precis. Alpha II antiplasmin deficiency is a rare autosomal recessive disorder leading to increased fibrinolysis and hemorrhage. We present a case report of a pregnancy complicated by this disorder. 1. Introduction Alpha II antiplasmin is a glycoprotein synthesized by the liver. It is present in two distinct forms: plasminogen binding and nonplasminogen binding. 20% of the plasminogen binding form is covalently linked to fibrin clots and renders them more resistant to lyses. It is inherited in an autosomal recessive fashion. A deficiency in its production allows the fibrin clots to lyse and dissolve prematurely resulting in hemorrhage. It is also a strong inhibitor of plasmin, an enzyme directly involved in fibrinolysis [1]. Plasmin is involved in the cleavage of extracellular vascular endothelial growth factor. A deficiency in its inhibitor (alpha II antiplasmin) has been shown in vitro mice studies to increase the mortality from an acute myocardial infarction. VEGF has been shown to increase the vascular permeability in ischemic tissue; its overproduction results in pulmonary edema and increased mortality. Elevated levels of VEGF have been associated with increased incidence of preeclampsia [2]. 2. Case Our patient first presented to our service at 10-week gestation. She was a 30-year-old Caucasian female Gravida 1, Para zero. She had been diagnosed with alpha II antiplasmin deficiency with a level 50% normal due to several episodes of severe hematuria secondary to nephrolithiasis. She had received multiple blood transfusions in her adolescence due to the severity of these bleeds. Her brother had been diagnosed with alpha II antiplasmin