HELLP Syndrome: MADE EASY

Ma. Victoria S. Valmonte-Torres, MD, FPOGS

Section of Maternal & Fetal Medicine & OB-GYNE Ultrasound Department of Obstetrics & Gynecology MCU-FDTMF Hospital

Purpose of the Lecture:

To address the most commonly asked practical questions about HELLP Syndrome.

What is HELLP Syndrome?

( Louis Weinstein, 1982 )

H - emolysis EL - elevated liver enzymes LP - low platelets

Pathophysiology of HELLP Syndrome

Inciting Agent to Insult (?)

Microvascular endothelial damage Intravascular platelet activation & deposition

Stimulates secretion of Thromboxane A2 & serotonin

Vasoconstriction & more platelet deposition / aggregation & damage to the blood vessel wall

Vasoconstriction & more platelet deposition / aggregation & damage to the blood vessel wall
Further vessel narrowing Circulating (serum) platelets Actual platelet count Hepatocellular Hypoxia

Hypertension

RBC damage Microangiopathic hemolysis LDH Hemoglobin

Hepatocellular & Periportal necrosis Liver enzyme Liver rupture

Is it a complication of preeclampsia?
Many authors consider it as a variant of preeclampsia, but it maybe a SEPARATE entity!

NOTE:
When preeclampsia is not present, diagnosis of the syndrome is often delayed!

How common is HELLP Syndrome?

HELLP Syndrome - 0.2 - 0.6% of all pregnancies Preeclampsia 5-7% of all pregnancies Superimposed HELLP occurs in 4-12% of preeclampsia / eclampsia

NOTE: The syndrome generally presents in the THIRD trimester of pregnancy During the postpartum period, the onset is typically within the first 48 hrs following delivery.

How Do We Diagnose HELLP Syndrome?

Diagnosis.

RISK FACTORS:
HELLP SyndromePreeclampsia
Multiparous Age > 25 y/o White Race Poor pregnancy outcome Nulliparous <20 or >45 y/o (+) FHx of preeclampsia DM, CHVD, TWINS

Diagnosis

Clinical Presentation:
Generalized malaise Epigastric pain Nausea and vomiting Headache 90% 65% 30% 31%

Diagnosis.

NOTE:
Because early diagnosis of this syndrome is critical, any pregnant woman who presents with malaise or a viral type illness in the 3rd trimester should be evaluated for HELLP Syndrome!

Diagnosis

Diagnostic Tests:
Hemolysis ( microangiopathic hemolytic anemia ) decreased hemoglobin/ hematocrit* increased LDH* decreased haptoglobin increased serum bilirubin PBS - schistocytes, burr cells ( damaged RBCs )

Diagnosis...

Diagnostic Tests ...

EL - Elevated Liver Enzymes
increased SGPT*

increased SGOT

Diagnosis...

Diagnostic Tests...
LP - Low Platelets count ( thrombocytopenia )
- earliest to appear - best indicator of HELLP syndrome

Diagnosis...

Therefore, the minimum laboratory tests youll request to diagnose HELLP Syndrome are:
Hgb / Hct LDH SGPT APC

Is HELLP Syndrome the same as DIC?

NO!
Because in HELLP the problem is solely platelet depletion . In DIC, other coagulation / clottimg factors are deranged.

Therefore, to differentiate, request for prothrombin time (PT) PTT fibrinogen levels
NORMAL HELLP Syndrome

Prolonged PT/PTT and decreased fibrinogen level ( < 300 mg/dl ) DIC

How do we classify HELLP Syndrome?

Mississippi Tennessee based on platelet count nadir 3 classes based on the number of abnormalities present complete or incomplete

Classification of HELLP...

Mississippi Classification:
Thrombocytopenia:
Class 1 - < 50,000 / ul Class 2 - 50,000 - 100,000 / ul Class 3 - > 100,000 - <150,000 / ul

Hemolysis / Liver dysfunction:

LDH - at least 600 IU/L SGPT- at least 40 IU/L

Classification of HELLP...

Tennessee Classification:
Complete HELLP
- < 100,000/ul platelets - LDH - at least 600 IU/L - SGPT - 70 IU/L

Incomplete HELLP
- Only one or two of the above is/are present

Management...

How do we manage HELLP Syndrome?

Antenatally? During delivery? In the postpartum period?

Algorithmic Management of HELLP Syndrome

 34 weeks Administer double-dose corticosteroids

Fetal monitoring (CTG, AFI, Doppler) Maternal clinical (S/Sxs) & Laboratory monitoring ( LDH, Hgb, SGPT, APC ) STABLE

> 34 weeks DELIVER

WORSENS

Conservative mx (Feto-maternal monitoring) Until fetal lungs mature

Continue monitoring / steroids until the laboratory parameters improve

DELIVER - Route? - Transfusion? - Postpartum management

Management...

Antenatally...
Maternal and Fetal Monitoring:
Maternal - clinical findings ( HPN, bleeding, etc.)
- laboratory tests: ( q 24 - 72 hours ) LDH, APC, SGPT, Hgb/Hct

Fetal - BPS, Doppler

Management...

Antenatally. Giving of Double-dose Dexamethasone

- 10 mg IV q 12 hours until delivery

Management ...

Antenatally Proven benefits of double -dose Dexamethasone:

1. Enhance fetal lung maturity 2. Improved feto-maternal outcome 3. Ameliorate the HELLP process ( APC, LDH/SGPT ) 4. Increased maternal urine output 5. Reduced need for blood/ blood products

Management...

DELIVERY... General Recommendation:

CS if
Class 1 HELLP Superimposed DIC AOG <32 weeks

Management...

Delivery...
Trial of labor if
Class 2 -3 or
Incomplete HELLP who are stable w/ favorable cervix and at least 32 wks AOG

Management...

Delivery.

Is platelet transfusion routine during delivery of patients with HELLP Syndrome?

Management...

Delivery NOTE:
HELLP patients with APC of more than 40,000/ ul are UNLIKELY to bleed.

Management...

Delivery...
Recommendation for intrapartum platelet transfusion ( at least 6 packs):
TRANSFUSE if APC is < 50,000 / ul ( CS ) or < 20,000 / ul ( NSVD )

Management...

Delivery...
What anesthesia should be given intrapartum?
AS a general rule, epidural block is recommended if the APC > 100,000/ul, otherwise, general anesthesia is given.

Management ...

Postpartum...
NOTE:
The laboratory abnormalities in HELLP typically worsen after delivery and then begin to resolve by 3-4 days postpartum Steroids given antenatally do not prevent the typical postpartum worsening of these HELLPrelated laboratory abnormalities

Management ...

Postpartum NOTE...
However, these laboratory abnormalities resolve more quickly in patients who continue to receive steroid postpartum. Should continue to give steroids until APC is > 100,000/ ul and LDH and SGPT decrease.

Management ...

Postpartum NOTE...

In case of worsening of laboratory abnormalities 3-4 days ff. delivery, plasma exchange transfusion using fresh frozen plasma is indicated.

Management

Postpartum NOTE...

Purpose of Plasma exchange transfusion:

To remove the debris from the hemolytic process and replace the depleted clotting factors

Counselling for future pregnancies:

Risk of Recurrent HELLP : 19-27 % Risk of developing Preeclampsia up to 43% in future pregnancies

Counselling...

NOTE:
Patients with Class 1 HELLP Syndrome have the highest risk of recurrence in future pregnancies.

Patients with atypical early-onset preeclampsia or HELLP Syndrome should be screened for APAS.

In Summary...
The main INCITING event leading to microvascular endothelial damage in HELLP Syndrome is still unknown. HELLP Syndrome maybe a SEPARATE entity to Preeclampsia

In summary...
High index of suspicion is given among multiparous, middle-aged pregnant patients with previous history of poor pregnancy outcome presenting with generalized malaise or viral type of illness in the third trimester of pregnancy.

In summary...
Minimum laboratory tests that maybe requested to diagnose the syndrome are LDH & Hgb/Hct ( hemolysis ) SGPT ( liver problem) APC ( thrombocytopenia )

PT, PTT, Fibrinogen levels might be requested to differentiate bleeding due to HELLP vs DIC.

In Summary...
Double-dose Dexamethasone - 10 mg IV q 12 hours, proved to improve the general course of the disease. Route of delivery? CS - if with complete/class 1 HELLP, (+) DIC, <32 weeks NSD - class 2-3 or incomplete HELLP who are stable with favorable cervix and at least 32 wks AOG

In Summary...
Intrapartum Transfusion? APC >40,000/ul - less likely to bleed! Transfuse if : CS NSD < 50,000/ul < 20,000/ul

In summary...
Anesthesia during delivery? Epidural block for APC >100,000/ul otherwise, general anesthesia

In summary...
There is expected worsening of the laboratory parameters postpartum, but a typical improvement 3-4 days ff. delivery.
Giving of double-dose Dexa should be continued post partum until APC is > 100,000/ul and LDH and SGPT decrease.

In summary...
In case of further worsening 3-4 days postpartum, plasma exchange transfusion is indicated. Recurrence rate of HELLP Syndrome is 19- 27 %. In cases of early onset atypical Preeclampsia /HELLP syndrome, APAS should be ruled out!

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