Preeclampsia in a factor XI deficient woman: discussion about one case

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A 33-year-old woman was hospitalized at the 34th week of a bichorionic biamniotic twin pregnancy following an in-vitro fertilization procedure using sperm and oocyte donation. Pastmedical history included a series of surgical procedures (breast adenofibroma, bilateral otosclerosis, tenosynovitis, breast volume reduction, and tooth extractions) without any bleeding problem. Her prepregnancy BMI was 28.4 kg/m2. No tendency for hypertension was previously noted. This pregnancy was uneventful until the occurrence of hypertension (SBP between 140 and 160 mmHg, DBP between 90 and 110 mmHg) associated with swelling (3 kg weight gain in 5 days) and reduced urine output. The hemogram showed: 10.8 g/dl hemoglobin (Hb), 7.2 G/l white blood cells with normal differential, and 165 G/l platelets. No schistocytes were seen on the blood film. The activated partial thromboplastin time (aPTT) ratio was 1.31. Factors (F) XII, IX, VIII, and von Willebrand were above 100%. Prothrombin time was 100%. FXI coagulant (FXIc) activity was decreased at 27% (assessed from two different samples taken on two consecutive days). Fibrinogen was 3.31 g/l. Fibrin monomer levels were normal. Serum sodium level was 129 mmol/l, protidemia 51 g/l (including 25.1 g/l serum albumin). Uric acid was elevated (68 mg/l) and a proteinuria (2.4 g/l) was noted. No significant impairment in liver or renal function and no elevated serum lactate dehydrogenase were evidenced. Two girls (2130 and 2000 g) were delivered by cesarean section at 35 weeks of gestation under general anesthesia. An about 1000 ml blood loss was observed (Hb: 8 g/dl, day +1). Thereafter, this woman developed a postcesarean hematoma associated with an Hb nadir at 7.2 g/dl 4 days after delivery. The biology did not show any additional sign suggestive of DIC. Six months after delivery, FXIc was 48% (aPTT ratio: 1.32). Familial inquiry revealed a partial (heterozygous) FXI deficiency in the patient’ mother (FXIc: 54%) and sister (FXIc: 49%).

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