18 | CBN September 2021

iNteractive quiz

Case 1 -
Dr Melanie D’Souza and Dr Marie M Salib
Chemical Pathology, ACT Pathology, Canberra, ACT
Email: melanie.f.dsouza@act.gov.au

Case History
31-year-old female at 33/40 (G1P0) gestation with twins presented with a
1-day history of lower limb oedema, nausea and vomiting and headache.
She was normotensive. Over the following day, she developed liver
function test (LFT) abnormalities and a reduction in her eGFR. A drop in
haemoglobin was noted, with a normal platelet count. Her initial laboratory
investigations are shown below.

Day 1 Day 2 Reference intervals

Bilirubin 46 48 2-20 µmol mol/L

ALT 435 415 <33 U/L
AST 235 235 <32 U/L
ALP 267 287 30-110 U/L
GTT 96 107 <56 U/L
Protein 53 55 60-80 g/L
Albumin 23 23 33-50 g/L
Creatinine 169 180 45-90 µmol/L
eGFR 34 32 >90

Hb 105 95 115-160 g/L

WCC 31 27 4-11 x109/L
Platelet 269 279 150-400 x109/L

Blood film on Day 2: Leukoerythroblastic blood film with anaemia with

occasional schistocytes, neutrophilia with toxic granulation but normal
platelet count.
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iNteractive quiz

Questions
1. What are the possible causes of abnormal LFTs in this patient?

2. What clinical and biochemical features support the possible

diagnoses? What other tests would be useful?

3. What is/are the best management option/s in this situation?

Answers on page 21
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iNteractive quiz

Case 1

Discussion

Question 1
The LFTs demonstrate moderate-severe hepatocellular dysfunction, as demonstrated by the significant elevation
of the serum aminotransferases compared to the alkaline phosphatase. In the third trimester of pregnancy this
raises concerns for Acute Fatty Liver of Pregnancy (AFLP) and Haemolysis, Elevated Liver Enzymes and Low
Platelets (HELLP) syndrome, however other considerations include cholestasis of pregnancy, viral hepatitis, Budd-
Chiari syndrome, and drug-induced liver injury. A rise in ALP is also expected in the third trimester due to placental
production of the enzyme.

Question 2
This case highlights the significant clinical and biochemical overlap that can occur in the setting of AFLP and HELLP.
Clinical presentation of both AFLP and HELLP is often non-specific with abdominal discomfort, nausea and general
malaise and has multisystem involvement. Rarely, features of acute hepatic failure may be present. In AFLP, the
LFT elevation is usually 5-10 times above the upper limit of normal with elevated bilirubin and ammonia levels. The
Swansea criteria is commonly used to assist diagnosis and liver biopsy is not usually required. In HELLP, key features
include haemolytic anaemia, thrombocytopaenia with LFTs raised >2 times the upper limit of normal. Coagulation
studies and urine protein: creatinine ratios may also be useful in assessing for end-organ damage. In this case, the
final diagnosis was AFLP with features of HELLP.

Swansea criteria for diagnosis of acute fatty liver of pregnancy

Six or more criteria required in the absence of another cause
Vomiting
Abdominal pain
Polydipsia/polyuria
Encephalopathy
Elevated bilirubin >14µmolL
Hypoglycaemia <4mmol/L
Elevated urate >340 µmol/L
Leucocytosis >11x109 cells/ L
Elevated transaminases (ASL or ALT) >42 U/L
Elevated ammonia >47µmol/L
Renal impairment; creatinine >150 µmol/L
Coagulopathy; prothrombin time >14s or APTT >34s
Ascites or bright liver on ultrasound
Microvesicular steatosis on liver biopsy Questions on page 19
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Question 3
Management of both AFLP and HELLP is urgent delivery despite gestational age and medical support of the mother.
There is an increased risk of both conditions in subsequent pregnancies and therefore women should be counselled
on the risk. Future pregnancies should occur in a highly specialised unit with close monitoring. In this case, an
emergency caesarean section was performed on Day 2 of admission resulting in the birth of two live male infants.
Over the subsequent days, LFTs, renal impairment and anaemia resolved with no further haemolysis seen on blood
film (see below follow up laboratory investigations).

Day 5 post-partum Reference intervals

Bilirubin 26 2-20 µmol/L

ALT 42 <33 U/L
AST 24 <32 U/L
ALP 163 30-110 U/L
GTT 133 <56 U/L
Protein 53 60-80 g/L
Albumin 26 33-50 g/L
Cr 57 45-90 µmol/L
eGFR >90 >90

Hb 110 115-160 g/L

WCC 13.5 4-11 x109/L
Platelets 463 150-400 x109/L

References
1. Liu, Joy; Ghaziani, Tara T; Wolf, Jacqueline L Acute Fatty Liver Disease of Pregnancy: Updates in Pathogenesis,
Diagnosis, and Management, American Journal of Gastroenterology 2017; 112 (6): 838-846.

2. Nelson, David B; Byrne, John J; Cunningham, F. Gary Acute Fatty Liver of Pregnancy, Obstetrics & Gynecology
2021;137 (3): 535-546

3. Lee, Richard; Reau, Nancy Acute Fatty Liver of Pregnancy. In: UpToDate, Lindor, Keith and Lockwood, Charles
(Eds) UpToDate, Waltham, MA. (Accessed on 05/08/2021).

4. Haram, K; Svendsen, E; Abildgaard, U The HELLP syndrome: Clinical issues and management. A Review. BMC
Pregnancy Childbirth 2009; 9(8).

5. Hammoud, G.M; Ibdah, J.A. Preeclampsia-induced Liver Dysfunction, HELLP syndrome, and acute fatty liver of
pregnancy. Clinical Liver Disease 2014; (4): 69-73.