Abstract
HELLP syndrome and the less common acute fatty liver of pregnancy (AFL) are unpredictable,
life-threatening complications of pregnancy. The similarities in their clinical and
laboratory presentations are often challenging for the obstetrician when making a
differential diagnosis. Both diseases are characterised by microvesicular steatosis
of varying degrees of severity. A specific risk profile does not exist for either
of the entities. Genetic defects in mitochondrial fatty acid oxidation and multiple
pregnancy are considered to be common predisposing factors. The diagnosis of AFL is
based on a combination of clinical symptoms and laboratory findings. The Swansea criteria
have been proposed as a diagnostic tool for orientation. HELLP syndrome is a laboratory
diagnosis based on the triad of haemolysis, elevated aminotransferase levels and a
platelet count < 100 G/l. Generalised malaise, nausea, vomiting and abdominal pain
are common symptoms of both diseases, making early diagnosis difficult. Clinical differences
include a lack of polydipsia/polyuria in HELLP syndrome, while jaundice is more common
and more pronounced in AFL, there is a lower incidence of hypertension and proteinuria,
and patients with AFL may develop encephalopathy with rapid progression to acute liver
failure. In contrast, neurological symptoms such as severe headache and visual disturbances
are more prominent in patients with HELLP syndrome. In terms of laboratory findings,
AFL can be differentiated from HELLP syndrome by the presence of leucocytosis, hypoglycaemia,
more pronounced hyperbilirubinemia, an initial lack of haemolysis and thrombocytopenia
< 100 G/l, as well as lower antithrombin levels < 65% and prolonged prothrombin times.
While HELLP syndrome has a fluctuating clinical course with rapid exacerbation within
hours or transient remissions, AFL rapidly progresses to acute liver failure if the
infant is not delivered immediately. The only causal treatment for both diseases is
immediate delivery. Expectant management between 24 + 0 and 33 + 6 weeks of gestation
is recommended for HELLP syndrome, but only in cases where the mother can be stabilised
and there is no evidence of foetal compromise. The maternal mortality rate for HELLP
syndrome in developed countries is approximately 1%, while the rate for AFL is 1.8 – 18%.
Perinatal mortality rates are 7 – 20% and 15 – 20%, respectively. While data on the
long-term impact of AFL on the health of mother and child is still insufficient, HELLP
syndrome is associated with an increased risk of developing cardiovascular, metabolic
and neurological diseases in later life.
Key words
HELLP syndrome - acute fatty liver of pregnancy - Swansea criteria - differential
diagnosis - treatment and prognosis
