Microangiopathic antiphospholipid antibody-associated syndrome in a pregnant lady

Abstract

We describe a gravid 37-year-old Chinese lady with known triple positive primary antiphospholipid syndrome with previous recurrent deep vein thrombosis and early spontaneous miscarriages. She was managed with low-molecular weight heparin, aspirin, hydroxychloroquine, prednisolone and monthly intravenous immunoglobulin. She presented with recurrent per-vaginal bleeding at 22 weeks of gestation and was found to have abruptio placentae. Anti-coagulation was held off. She subsequently delivered a stillborn at 24 weeks and anti-coagulation was restarted. Day 5 post-delivery, she developed HELLP, with hemolytic anaemia (Hb 10.1 g/dL, haptoglobin <30 g/L, LDH 2206 U/L), elevated transaminases (AST 1196 U/L, ALT 1130 U/L) and thrombocytopenia (platelet 28 × 10^9/L). There were also episodes of acute severe headache and abdominal pain assessed to be secondary to microvascular ischemia as CT did not reveal any thrombosis. Her blood pressure hovered persistently above systolic 180 mmHg, and required at least three anti-hypertensives. These were coupled with a new onset proteinuria of 2 to 3 g/day. There was no evidence of disseminated intravascular coagulation. She was assessed to have microangiopathic antiphospholipid syndrome and was started on plasmapheresis. On Day 10 post-partum, the patient complained of foul-smelling vaginal discharge and was found to have retained products of conception, which was immediately evacuated. Her course was followed by poly-microbial sepsis secondary to Enterococcus fecalis, Klebsiella Pneumoniae and Escherichia coli. The patient was further treated with imipenem and she completed eight exchanges of plasmapheresis followed by five days of intravenous immunoglobulins with good clinical and biochemical improvement.