HELLP syndrome

Question 1

How is HELLP syndrome defined?

Answer 1

HELLP syndrome is defined by a series of laboratory dysfunction including:
-Hemolysis (see schistocytes on peripheral smear, also see increased LDH >600 and indirect bilirubin)
-Elevated Liver enzymes - >70, twice the upper limit of normal
Low platelet - <100

***This is the Tennessee Classification

Question 2

What are maternal risks from HELLP syndrome?

Answer 2

• DIC in 21%
• Abruption in 16%
• Renal failure in 8%
• Stroke
• Venothromboembolism
• Need for c-section

Question 3

What are fetal risks from HELLP syndrome?

Answer 3

Prematurity, abruption and the risks associated with that.

Question 4

What would make you suspect a subcapsular hematoma?

Answer 4

• RUQ pain (occurs in 90%) and abnormal LFT test

- Nausea/vomiting (occurs in 50%)

Question 5

If a subcapsular hematoma is identified, how will you manage the patient?

Answer 5

• Obtain CT/MRI
• Assess hemodynamic and coagulation parameters
• Do not delay delivery

Question 6

What are the risks of a subcapsular hematoma?

Answer 6

• Liver rupture and failure
• Hemorrhage
• Maternal death (mortality occurs in 18-86% of patient with liver rupture)

***Care with patient transport to avoid rupture

Question 7

How do you manage a patient with a ruptured subcapsular hematoma?

Answer 7

• Call for massive transfusion protocol
• STAT anesthesia and trauma consultation
• Delivery via cesarean section, avoid further trauma to liver
• Care with patient transport to avoid further damage
• Trauma surgery consultation for peritoneal packing/hemostatic mesh
• PT/PTT/Fibrinogen/CBC
• Transfuse as needed

Question 8

How do you counsel patient about recurrence risks following a pregnancy complicated by HELLP syndrome?

Answer 8

Recurrence risk is 5%

Question 9

What is atypical HELLP and how often is it seen?

Answer 9

Seen in 15% and lacks HTN or proteinuria

Question 10

What laboratory findings in HELLP suggest an increase in maternal mortality?

Answer 10

AST greater than 2000
LDH more than 3000

Obtain blood work every 12 hours in patients with HELLP

Question 11

HUS verses Atypical HUS

Answer 11

HUS 
Hemolysis 
Uremia 
Thrombocytopenia 
Syndrome 
Caused by shiga toxin from E. Coli H7:0157

Atypical HUS can be caused by HELLP.
Hallmark is abnormal complement activation.

Treat with Soliris (anti C5 antibody - eculizumab)

Question 12

TTP

Answer 12

Thrombotic
Thrombocytopenic
Purpura
Hallmark - hemolysis, urea , thrombocytopenia, fever, altered mental state

See deficiency in ADAMS13

Treat with plasmaphoresis (plasma exchange)

Question 13

Acute Fatty Liver

Answer 13

1 in 10,000
Increase risk with LCHAD deficiency
\+/- hemolysis 
\+/- thrombocytopenia 
Increased bilirubin and AST and AMMONIA 
Treatment is DELIVERY and supportive care 
Lactulose to bring down ammonia