HELLP Syndrome:HELLP Syndrome:MADE EASYMADE EASY

Ma. Victoria S. Valmonte-Torres, MD, FPOGSMa. Victoria S. Valmonte-Torres, MD, FPOGSSection of Maternal & Fetal Medicine &Section of Maternal & Fetal Medicine &

OB-GYNE UltrasoundOB-GYNE UltrasoundDepartment of Obstetrics & GynecologyDepartment of Obstetrics & Gynecology

MCU-FDTMF HospitalMCU-FDTMF Hospital

Purpose of the Lecture:Purpose of the Lecture:

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

What is HELLP Syndrome?What is HELLP Syndrome?( Louis Weinstein, 1982 )( Louis Weinstein, 1982 )

• HH - - emolysisemolysis• ELEL - elevated liver enzymes- elevated liver enzymes• LPLP - low platelets- low platelets

Pathophysiology Pathophysiology of of

HELLP SyndromeHELLP Syndrome

Inciting Agent to Insult (?)Inciting Agent to Insult (?)

Microvascular endothelial damageMicrovascular endothelial damage

Intravascular platelet activation & depositionIntravascular platelet activation & deposition

Stimulates secretion ofStimulates secretion ofThromboxane A2 & serotoninThromboxane A2 & serotonin

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

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

HypertensionHypertension

↓ ↓ ActualActualplatelet countplatelet count

↓ ↓ CirculatingCirculating(serum) platelets(serum) platelets

Further vessel narrowing

RBCdamage

HepatocellularHepatocellularHypoxiaHypoxia

Hepatocellular &Hepatocellular &Periportal necrosisPeriportal necrosis

↑ Liver enzyme

Liver rupture

Microangiopathichemolysis

↑LDH↓Hemoglobin

Is it a complication of preeclampsia?Is it a complication of preeclampsia?

• Many authors consider it as a variant of preeclampsia, but it maybe a SEPARATE entity!

NOTE:NOTE:

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

How common is HELLP Syndrome?How common is HELLP Syndrome?

HELLP Syndrome - 0.2 - 0.6% of all pregnanciesPreeclampsia - 5-7% of all pregnancies

Superimposed HELLP occurs in 4-12% of preeclampsia / eclampsia

NOTE:NOTE:

The syndrome generally presents in the The syndrome generally presents in the THIRD trimester of pregnancyTHIRD trimester of pregnancy

During the postpartum period, the onset is During the postpartum period, the onset is typically within the first 48 hrs following typically within the first 48 hrs following delivery.delivery.

How Do We Diagnose How Do We Diagnose HELLP Syndrome?HELLP Syndrome?

Diagnosis….Diagnosis….RISK FACTORS:RISK FACTORS:

HELLP SyndromeHELLP SyndromePreeclampsiaPreeclampsiaMultiparousMultiparous NulliparousNulliparousAge > 25 y/oAge > 25 y/o <20 or >45 y/o<20 or >45 y/oWhite RaceWhite Race (+) FHx of preeclampsia(+) FHx of preeclampsiaPoor pregnancy outcomePoor pregnancy outcome DM, CHVD, TWINSDM, CHVD, TWINS

Diagnosis…Diagnosis…

Clinical Presentation:Clinical Presentation:

Generalized malaise -Generalized malaise - 90%90%Epigastric pain -Epigastric pain - 65%65%Nausea and vomiting -Nausea and vomiting - 30%30%Headache -Headache - 31%31%

Diagnosis….Diagnosis….

NOTE:NOTE:

• Because early diagnosis of this syndrome is Because early diagnosis of this syndrome is critical, any pregnant woman who presents critical, any pregnant woman who presents with with malaise or a viral typemalaise or a viral type illness in the illness in the 3rd trimester3rd trimester should be evaluated for should be evaluated for HELLP Syndrome!HELLP Syndrome!

Diagnosis…Diagnosis…

Diagnostic Tests:Diagnostic Tests:

HemolysisHemolysis( microangiopathic hemolytic( microangiopathic hemolytic anemia )anemia )

• decreased hemoglobin/ hematocrit*• increased LDH* • decreased haptoglobin• increased serum bilirubin• PBS - schistocytes, burr cells ( damaged

RBCs )

Diagnostic Tests ...

EL - Elevated Liver Enzymes increased SGPT* increased SGOT

Diagnosis...Diagnosis...

Diagnostic Tests...Diagnostic Tests...

Diagnosis...Diagnosis...

LP LP - Low Platelets count - Low Platelets count ( thrombocytopenia )( thrombocytopenia )

- - earliest to appearearliest to appear- best indicator of HELLP - best indicator of HELLP

syndromesyndrome

Therefore, the minimum laboratory Therefore, the minimum laboratory tests you’ll request to diagnose tests you’ll request to diagnose HELLP Syndrome are:HELLP Syndrome are:

Hgb / HctHgb / HctLDHLDHSGPTSGPTAPCAPC

Diagnosis...Diagnosis...

Is HELLP Syndrome the same as DIC?Is HELLP Syndrome the same as DIC?

NO!NO!

Because in Because in HELLPHELLP the problem is solely the problem is solely platelet depletionplatelet depletion . .

In In DICDIC, other , other coagulation / clottimg factorscoagulation / clottimg factors are are deranged.deranged.

Therefore, to differentiate, request for prothrombin time (PT)

PTTfibrinogen levels

NORMAL HELLP Syndrome

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

How do we classify HELLP Syndrome?How do we classify HELLP Syndrome?

Mississippi -Mississippi - based on platelet count nadirbased on platelet count nadir -- 3 classes3 classes

Tennessee -Tennessee - based on the number of based on the number of abnormalities presentabnormalities present-- complete or incompletecomplete or incomplete

Mississippi Classification:Mississippi Classification:

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

Hemolysis / Liver dysfunction:Hemolysis / Liver dysfunction:LDH - at least 600 IU/LLDH - at least 600 IU/LSGPT- at least 40 IU/LSGPT- at least 40 IU/L

Classification of HELLP...

Tennessee Classification:Tennessee Classification:

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

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

Classification of HELLP...

How do we manage HELLP How do we manage HELLP Syndrome?Syndrome?

• Antenatally?Antenatally?• During delivery?During delivery?• In the postpartum period?In the postpartum period?

Management...Management...

Algorithmic ManagementAlgorithmic Managementofof

HELLP SyndromeHELLP Syndrome

> 34 weeks> 34 weeks≤ ≤ 34 weeks34 weeks

DELIVERDELIVERAdminister double-dose corticosteroidsAdminister double-dose corticosteroids

Maternal clinicalMaternal clinical (S/Sx’s)(S/Sx’s)&&

Laboratory monitoringLaboratory monitoring( LDH, Hgb, SGPT, APC )( LDH, Hgb, SGPT, APC )

Fetal monitoringFetal monitoring(CTG, AFI, Doppler)(CTG, AFI, Doppler) STABLESTABLE

WORSENSWORSENS

DELIVERDELIVER- Route?- Transfusion?- Postpartum management

Continue monitoring / steroidsuntil the laboratory parameters

improve

Conservative mxConservative mx(Feto-maternal(Feto-maternal

monitoring)monitoring)Until fetal lungsUntil fetal lungs

maturemature

Antenatally...Antenatally...

Management...Management...

Maternal and Fetal Monitoring:Maternal and Fetal Monitoring:

MaternalMaternal - clinical findings ( HPN, bleeding, etc.) - clinical findings ( HPN, bleeding, etc.)- laboratory tests: ( q 24 - 72 hours ) - laboratory tests: ( q 24 - 72 hours )

LDH, APC, SGPT, Hgb/HctLDH, APC, SGPT, Hgb/Hct

FetalFetal - BPS, Doppler - BPS, Doppler

Antenatally….

Giving of “ Double-dose Dexamethasone”- 10 mg IV q 12 hours until delivery

Management...Management...

Antenatally…Antenatally…Proven benefits of double -dose Dexamethasone:

1. Enhance fetal lung maturity2. Improved feto-maternal outcome3. Ameliorate the HELLP process

( ↑ APC, ↓ LDH/SGPT )4. Increased maternal urine output5. Reduced need for blood/ blood products

Management ...Management ...

DELIVERY...DELIVERY...

General Recommendation:General Recommendation: CS if …CS if …

Class 1 HELLP Class 1 HELLP Superimposed DICSuperimposed DIC AOG <32 weeksAOG <32 weeks

Management...Management...

Delivery...Delivery...

Trial of labor if…Trial of labor if…Class 2 -3 orClass 2 -3 or

Incomplete HELLP who are stable w/ Incomplete HELLP who are stable w/ favorable cervix and at least 32 wks AOGfavorable cervix and at least 32 wks AOG

Management...Management...

Delivery….Delivery….

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

Management...Management...

Delivery…Delivery…

NOTE:NOTE: HELLP patients with APC of more than

40,000/ ul are UNLIKELY to bleed.

Management...Management...

Delivery...Delivery...

Recommendation for intrapartum platelet Recommendation for intrapartum platelet transfusion ( at least 6 packs):transfusion ( at least 6 packs):

TRANSFUSE if APC is < 50,000 / ulTRANSFUSE if APC is < 50,000 / ul

( CS ) or < 20,000 / ul ( NSVD )( CS ) or < 20,000 / ul ( NSVD )

Management...Management...

Delivery...Delivery...

What anesthesia should be given What anesthesia should be given intrapartum?intrapartum?

AS a general rule, AS a general rule, epidural blockepidural block is is recommendedrecommended if the APC > 100,000/ul, if the APC > 100,000/ul, otherwise, otherwise, general anesthesiageneral anesthesia is given.is given.

Management...Management...

Postpartum...Postpartum...

NOTE:NOTE:•The laboratory abnormalities in HELLP The laboratory abnormalities in HELLP

typically worsen after delivery and then begin typically worsen after delivery and then begin to resolve by 3-4 days postpartumto resolve by 3-4 days postpartum

•Steroids given antenatally Steroids given antenatally do notdo not prevent the prevent the typical postpartum worsening of these HELLP- typical postpartum worsening of these HELLP- related laboratory abnormalities related laboratory abnormalities

Management ...Management ...

Postpartum…Postpartum…

NOTE...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 ...Management ...

Postpartum…Postpartum…NOTE...NOTE...

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

Management ...Management ...

Postpartum…Postpartum…NOTE...NOTE...

Purpose of Plasma exchange transfusion:Purpose of Plasma exchange transfusion:

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

ManagementManagement

Counselling for future pregnancies:Counselling for future pregnancies:

Risk of Recurrent HELLP : 19-27 %Risk of Recurrent HELLP : 19-27 %

Risk of developing PreeclampsiaRisk of developing Preeclampsia

up to 43% in future pregnanciesup to 43% in future pregnancies

NOTE:NOTE:

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

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

Counselling...Counselling...

In Summary...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...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...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...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 weeksNSD - class 2-3 or incomplete HELLP

who are stable with favorable cervix and at least 32 wks AOG

In Summary...In Summary...

•Intrapartum Transfusion?

•APC >40,000/ul - less likely to bleed!

•Transfuse if :CS - < 50,000/ulNSD - < 20,000/ul

In summary...

•Anesthesia during delivery?

•Epidural block for APC >100,000/ul otherwise, general anesthesia

In summary...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 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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