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

Abstract

As proposed criteria (Swansea criteria) for the diagnosis of acute fatty liver of pregnancy (AFLP) do not include antithrombin (AT) activity, diagnosis of AFLP may be delayed. The aim of this review is to underscore problems in the differential diagnosis of AFLP and the syndrome of hemolysis, elevated liver enzymes and low platelet counts (HELLP syndrome) and to facilitate prompt diagnosis of AFLP. Published works dealing with liver dysfunction in pregnancy, HELLP syndrome and AFLP were reviewed. AFLP and HELLP syndrome shared common clinical, laboratory, histological and genetic features, and differential diagnosis between them was often difficult. However, HELLP syndrome was likely to occur in patients with hypertension, but AFLP occurred often in the absence of hypertension. In addition, AFLP was exclusively associated with pregnancy‐induced antithrombin deficiency (PIATD). Approximately 50% of patients with AFLP did not have thrombocytopenia at presentation. As the Swansea criteria for AFLP did not include PIATD, diagnosis of AFLP was delayed until manifestation of life‐threatening complications; 60% of women were admitted to intensive care and 15% to a specialist liver unit. In conclusion, incorporation of AT activity of less than 65% into the diagnostic criteria for AFLP may facilitate suspicion and prompt diagnosis of AFLP, decrease uncertainty regarding the diagnosis of AFLP, and contribute to better investigation and understanding of the process leading to the development of liver dysfunction.

Isolated pregnancy‐induced anti‐thrombin deficiency in a woman with twin pregnancy

“…More than 60% of cases of PIATD occur, however, in the absence of HDP, similar to the lack of preceding HDP in approximately half of AFLP patients . In addition, half of AFLP patients lack thrombocytopenia at presentation . Therefore, although neither HDP nor thrombocytopenia was present initially in this patient, the present patient was carefully monitored and found to have depressed AT activity, with a decrease to 41% of normal, concomitant with the development of pre‐eclampsia 8 days prior to delivery.…”

“…Given that HELLP syndrome is likely to occur in patients with pre‐eclampsia, physicians may monitor platelet count in such cases. More than 60% of cases of PIATD occur, however, in the absence of HDP, similar to the lack of preceding HDP in approximately half of AFLP patients .…”

“…Hemolysis/ elevated liver enzyme/ low platelet (HELLP) syndrome is a life‐threatening complication diagnosed in pregnant women with elevated serum lactate dehydrogenase (LDH) and aspartate aminotransferase (AST) and low platelet count. Given that women with hypertensive disorders of pregnancy (HDP) are likely to develop HELLP syndrome, laboratory work‐up including blood variables, such as platelet count, AST, and LDH, is recommended in such cases . Some patients with elevated AST and LDH, however, lack low platelet count in the presence of severely depressed anti‐thrombin (AT, also termed AT III) activity level, designated as pregnancy‐induced AT deficiency (PIATD), which can precede the clinical manifestation of acute fatty liver of pregnancy (AFLP) …”

Outcomes and risk factors of patients with acute fatty liver of pregnancy: a multicentre retrospective study

“…There is disagreement in the published literature regarding the symptoms and clinically common manifestations reported, in general, which includes malaise, anorexia, nausea, vomiting, abdominal pain, jaundice, ascites, hypertension and abdominal distension. (4,(12)(13)(14)17) We found that anorexia, nausea, vomiting and abdominal pain were the main prodromic symptoms while progressive jaundice and ascites were common signs.…”

“…(10) Simultaneously, neonatal mortality rate has also decreased, which is in the range of 7% to 66%. (3,11) Previous clinical studies on AFLP, largely based on a few patients due to its low prevalence, have found large variations in results vis-à-vis its epidemiology, (2)(3)(4)(5)(6) symptoms, (12) complications (12) and outcomes. (3,10,11,13) For instance, Cheng et al (14) found that, besides aetiology, other clinical characteristics of pregnancy (e.g.…”