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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