Severe Acquired Antithrombin III Deficiency in Relation to Hepatic and Renal Insufficiency and Intrauterine Fetal Death in Late Pregnancy

Abstract

Acute fatty liver (AFLP) is a rare complication of late pregnancy. The maternal and fetal mortality has earlier been reported to be about 75 %, but during the last decade a reduced mortality to about 30 and 50%, respectively, has been reported in the literature. Disseminated intravascular coagulation (DIC) has been suggested as a contributing cause to the high mortality. The treatment of DIC has long been under debate, and recently the administration of antithrombin III (AT) concentrate in addition ot other supportive treatment has been reported successful. This paper presents the survival of 1 patient with severe liver and renal failure indicating AFLP complicated by severe disturbances in blood coagulation and fibrinolysis. The patient was treated with AT concentrate and small doses of heparin, blood coagulation factors, large amounts of glucose intravenously and supportive intensive care. The pregnancy was terminated by cesarean section. The child was stillborn and 75% of the placental parenchyma was fibrosed.

Differentiation of acute fatty liver of pregnancy from syndrome of hemolysis, elevated liver enzymes and low platelet counts

“…Thrombocytopenia is exclusively seen in HELLP syndrome, while varied platelet counts were seen in AFLP (Table ). The mean or median value for platelet count at presentation ranged from 123 × 10 9 /L to 151 × 10 9 /L, while AT activity was exclusively and severely depressed, ranging 0.0–69% when tested (Table ) . However, two prospective studies to determine the incidences of liver dysfunction and AFLP conducted in the UK in 1999 and 2005 using the Swansea criteria for diagnosis of AFLP (Table ) did not determine AT activity or query status of AT activity .…”

Liver dysfunction in triplet pregnancies: Relation to antenatal changes in antithrombin activity and platelet count

“…Both conditions are likely to be complicated with reduced AT activity and/or thrombocytopenia. [5][6][7]9,[18][19][20] Taking changes in blood variables related to hemostasis, coagulation and fibrinolysis, such as platelet count, fibrinogen, D-dimer and AT activity, into account, five of the six women with liver dysfunction (Cases 1-5 in Table 2 and Fig. 2) may have been those with either AFLP or HELLP syndrome.…”

“…However, reduced AT activity is frequently associated with acquired bleeding diathesis or disseminated intravascular coagulation . Its activity ranges from 80% to 130% in healthy subjects, whereas acquired AT deficiency is frequently seen in patients who are admitted to the intensive care unit, including patients with AFLP …”

“…4 Its activity ranges from 80% to 130% in healthy subjects, whereas acquired AT deficiency is frequently seen in patients who are admitted to the intensive care unit, 4 including patients with AFLP. [5][6][7][8][9][10][11][12] Thrombocytopenia is seen in patients with HELLP syndrome. 13 Some women exhibit gradual decline in AT activity during pregnancy [14][15][16] and gradual decreases in AT activity (pregnancy-induced antithrombin deficiency [PIATD]) and/or platelet count (gestational thrombocytopenia) were suggested to precede the development of liver dysfunction in these complications.…”

Association of antenatal antithrombin activity with perinatal liver dysfunction: A prospective multicenter study

“…As consumption coagulopathy leading to disseminated intravascular coagulation may be a major problem and as baseline abnormalities on laboratory data at presentation may be associated with outcome in women with HELLP syndrome and AFLP, early suspicion may be important to improve outcome of these patients . In a series of case reports and reports on case series regarding AFLP patients, reduced antithrombin (AT) activity was seen in all cases tested . In addition, a gradual decline in AT activity was documented before the development of liver dysfunction, suggesting a close relationship between AT deficiency and AFLP …”