HELLP

SYNDROME
OUTLINE
z
CASE PROFILE

DIFFERENTIAL DIAGNOSISIS

DEFINITION

CLASSIFICATION

PATHOGENESIS

MANIFESTATIONS

MANAGEMENT

COMPLICATIONS
GENERAL
z DATA

 29 YO

 G1P0

 ODW

 Roman Catholic
z

PAST MEDICAL HISTORY

 BIPOLAR disorder since 16yo

 maintained on Risperidone 2mg/tab, 1 tab OD

 No hypertension

 No diabetes

 No asthma

 No previous surgery

 s/p Laminectomy for Cauda Equina Syndrome sec to HNP

(L5-S2) – 2013
z

FAMILY MEDICAL HISTORY

 No hypertension

 No diabetes

 No asthma

 No malignancy

 No thyroid disorder

 No family history of Bipolar disorder

z

PERSONAL AND SOCIAL

HISTORY
 College graduate

 Housewife

 Non-smoker

 Non-alcohol beverage drinker

 No eating disorder
z

MENSTRUAL HISTORY
 M – 14

 I – Regular

 D – 3-4 days

 A – 3-4 Pads/day

 S – (-) Dysmenorrhea
z

Gynecologic HISTORY
 Coitarche: 25 y/o

 1 sexual partner

 (-) STD, dyspareunia, post-coital bleeding

 z

 (+) cognizant of pregnancy 7 weeks

PRENATAL AOG

HISTORY  (+) difficulty of sleeping

1st  (-) Hypogastric pain

TRIMESTER  (-) Vaginal bleeding

 (-) epistaxis

 (-) gum bleeding

 (-) skin lesions

  LAB WORK-UP:
 CBC with QPC – normal
z
 Urinalysis – normal
 HBsAg - nonreactive
PRENATAL  RPR – nonreactive
TRANSVAGINAL UTZ (3/3/2020)
Twin live intrauterine gestation
HISTORY 7 weeks 4 days, and 7 weeks and 5
days by CRL
1st Both with good cardiac activities

TRIMESTER
Single gestational sac, 2 yolk sac
 Medications: consistent with monochorionic,
diamniotic gestation
 Multivitamins 1 tab OD Normal ovaries

 Folic acid 1 tab OD

 Calcium 1 tab BID
 z

PRENATAL  Due to history of Bipolar disorder, patient also

HISTORY sought consult with her Psychiatrist
1st  Advised to continue taking

TRIMESTER  Risperidone 2mg OD

 (PREGNANCY CATEGORY C)

 Valproic acid 500mg once a month

 (PREGNANCY CATEGORY X)
 z
 (+) good fetal movement

 (-) uterine contractions

PRENATAL
 (+) difficulty of sleeping
HISTORY
2nd  (-) epistaxis

TRIMESTER  (-) gum bleeding

 (-) skin lesions

 75g OGTT (26 4/7 weeks AOG)
z FBS 72mg/dl
1st hour 167mg/dl
2nd hour 151mg/dl
PRENATAL 2nd trimester scan (6/27/2020)
HISTORY Pregnancy uterine, live

2nd Twin A – cephalic, 23 weeks 6 days

AOG, EFW 645g AGA

TRIMESTER Twin B – breech presentation, 23 wks 6

days AOG EFW: 677g AGA
Medications:
1. FeSO4 1 tab OD
2. Folic acid 1 tab OD
3. Calcium 1 tab BID
4. Risperidone 2mg/tab, 1 tab OD
5. Valproic acid 500mg once a month
  (+) Good fetal movement
z
 (+) difficulty of sleeping

 (-) Headache
PRENATAL
 (-) Epigastric pain
HISTORY
 (+) occasional uterine contractions
3rd
TRIMESTER  (+) palmar erythema

 (+) bipedal edema

Medications:
1. FeSO4 1 tab OD
2. Folic acid 1 tab OD
3. Calcium 1 tab BID
4. Risperidone 2mg/tab, 1 tab OD
 (At 31 weeks AOG)
z

 (+) Good fetal movement

PRENATAL  (+) difficulty of sleeping

HISTORY  (-) Headache
3rd  (-) Epigastric pain
TRIMESTER  (+) Occasional uterine contractions

 (+) palmar erythema

 (+) bipedal edema

 Sought consult at private OB-GYN

 z Managed as
Threatened preterm labor
Work-up:
PRENATAL × CBC with QPC - normal
HISTORY × Urinalysis - normal
2nd
TRIMESTER Meds:
1. Isoxuprine 10mg 1 tab TID x 7 days
2. Betamethasone 12mg TIM q12H x 2
doses - completed
 z Latest ultrasound (19 Aug):
Twin Pregnancy, placenta grade II-III
Twin A: 32 3/7 weeks AOG
PRENATAL EFW: 1827 +/- 267 grams
Transverse lie
HISTORY AFI: 10 cm
2nd BPS: 8/8

TRIMESTER Twin B: 31 6/7 weeks AOG

EFW: 1838 +/- 268 grams
Cephalic
AFI: 12 cm
BPS: 8/8
PHYSICAL EXAMINATION
GEN SUR Conscious, coherent, not in cardiorespiratory distress

BP:120/90 mmHg HR: 98 bpm RR:20 cpm T:36.8 ‘C

VS
Wt 65 kg Ht: 5’4 BMI 21.9KG/M 2

HEENT Anicteric sclerae, pink palpebral conjunctivae, no nasoaural discharge

HEART Adynamic precordium, normal rate regular rhythm, no murmur

(+) Globular
ABDOMEN FH: 33cm
FHT1: 140s bpm FHT2: 150s bpm

SE Cervix is pinkish, no polyp, no erosion with minimal whitish discharge,

EXT Full and equal pulses, (+) bilateral, Palmar erythema, (+) Grade III bipedal edema
 ADMITTING DIAGNOSIS:
z G1P0 Twin Pregnancy uterine 32 5/7
weeks AOG, Transverse-cephalic,
Monochorionic-diamniotic, not in labor;
Bipolar Disorder
 CBC 8/25 UA 8/25 8/25
z HGB 101 Color Y Na 136.9
HCT 0.28 Trans ST Cl 100
RBC 3.11 Ph 7.0 Ica 1.13
WBC 9.69 sp G 1.010 Img 0.42
SEG 0.74 Sugar - Crea 42.2
LABORATORY LYM 0.013 Protein - BUN 2.71

WORK-UP EOS
MON
0.01
0.12
RBC
Pus
0
0-7
SGOT
SGPT
396.5
463.5
BAS 000 Bac   LDH 357
MCV 90 EC 14 ALB 33.7
MCH 32 Crystal  
MCHC 35.80 MT  
RDW 13 AU  
PLT 182 Ketones  
 REVISED DIAGNOSIS:
G1P0 Twin Pregnancy uterine 32 5/7 weeks
AOG, Transverse-cephalic, Monochorionic-
diamniotic, not in labor; Pre-eclampsia
z T/C HELLP syndrome;
R/O Liver pathology;
T/C Iron Deficiency Anemia;
Bipolar Disorder
  29 YO
z
 G1P0
 32 4/7 weeks AOG
 Twin pregnancy, monochorionic-diamnionic
SALIENT  Diagnosed with Bipolar disorder maintained on
FEATURES Valproate and Risperidone
 (+) difficulty sleeping
 (+) Bipedal Edema
 (+) palmar erythema
 No history of BP elevations
 (+) Grade III Bipedal edema
 Low hemoglobin
 Elevated liver enzymes
 z Diagnosis:
T/C HELLP Syndrome
t/c Acute Fatty Liver of Pregnancy
t/c Viral Hepatitis

Referred to Plan:
• For repeat platelet count, SGOT,SGPT, Crea,
Perinatologist LDH
• For Serum bilirubin, Amylase, CT, BT, PT, PTT,
ABG, Serum ferritin, TIBG, Hepatitis profile
• For HBT Utz, Chest xray w/ abdominal shield
 z Diagnosis:
Probably secondary to drug induced (the long
Referred to term use od ant0psychotic drug with
hepatotoxic effect)
GI service Cannot totally rule out HELLP syndrome
r/o Viral Hepatitis
re: Elevated Plan:
Liver • For Hepatitis profile
• For HBT Ultrasound
Enzymes • For ALP, TB, PTT, INR
 z Diagnosis:
Insomnia secondary to Medical condition

Plan:
• Non-pharmacological management
Referred to • Sleep hygiene
• Breathing exercises
DPBS
 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY

VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY

DRUG INDUCED
Laboratory results: HGB
8/25
101
8/26

HCT 0.28
HBT Ultrasound: PLT 182 173
SGOT 398 372
No Pathologic SGPT 463 443
Findings LDH 383
Crea 50 45.1
Bilirubin 5.0
Chest xray: Dir bilirubin 2.9
Indi Bilirubin 2.1
No significant Ferritin 62.3
chest findings PT 12.2
INR 0.96
APTT 21.9
24H urine protein (29 Sept) % act 108.5
301 grams
 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY

VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY

DRUG INDUCED
 z ACUTE FATTY LIVER OF
ELEVATED PREGNANCY
LIVER G1 
✔️ Common in Primigravid

ENZYME Twin Pregnancy


✔️ Common in multiple pregnancy ✔️
❌


❌ Manifest during 3rd trimester of
pregnancy with nausea, malaise
and anorexia
G1

❌ Hx of Hepatitis C

❌ Hx of OCP use🆕

❌ Hx of pruritus
Normal results

❌ Elevated bile acid level, bilirubin,

prolonged bleeding parameters

 ACUTE
z FATTY LIVER OF PREGNANCY

• rare, potentially life-threatening, pregnancy-related disease that affects 1 in 7000 to 16,000

pregnancies

• Maybe due to fatty acid oxidation disorders

• S/Sx: nausea, malaise, anorexia, polydipsia, polyuria

• Lab: High levels of bilirubin, creatinine, uric acid, and neutrophils; a prolonged prothrombin time;
acidosis; and hypoglycemia.

• Difficult to diff with HELLP

• Imaging: CT scan
 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY
❌
VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY

DRUG INDUCED
 z
ELEVATED VIRAL HEPATITIS

LIVER
ENZYME 
❌ Hx of Hepatitis infection

✔️ Has non-reactive result
 Pending hepatitis profile

❌ No history of blood transfusion

✔️ Has hepatitis vaccination


 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY
❌
❌
VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY

DRUG INDUCED
 z
INTRAHEPATIC CHOLASTASIS
ELEVATED OF PREGNANCY
LIVER
ENZYME ✔️ Common in multiple pregnancy


❌ Hx of Hepatitis C


❌ Hx of OCP use
❌

❌ Hx of pruritus


❌ Elevated bile acid level
 INTRAHEPATIC
z CHOLASTASIS OF PREGNANCY

 affects 0.7% of white pregnant women as compared to Southeast Asian women

 Cause is complex, with genetic, endocrine, and environmental factors playing roles
 Evidence found among sisters
 Elevated repro hormone (ie multiple preg)
 Those with Hepa C

 Usually presents with pruritus

 Lab: elevated liver enzymes and bile acid

 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY
❌
❌
VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY
❌
DRUG INDUCED
 z
ELEVATED DRUG INDUCED
LIVER
ENZYME  Risperidone
 No liver toxicity

 Valproic acid
 No liver toxicity
 z
ELEVATED
HELLP SYNDROME
LIVER
ENZYME ACUTE FATTY LIVER OF
PREGNANCY
❌
❌
VIRAL HEPATITIS

INTRAHEPATIC CHOLASTASIS OF
PREGNANCY
❌
❌
DRUG INDUCED
Course in the ward
Serial SGOT and SGPT
(+) Headache CBC 8/25 8/28 8/29
HGB 101
(+) Epigastric pain HCT 0.28
PLT 182 197 172
(+) Grade III bipedal
edema 8/25 8/26 8/27 8/28 8/29
SGOT 398 372 337 339 346
SGPT 463 443 365 386 360
LDH 182 357 383
BP ranges:
100-140/80-90 24H urine protein (29 Sept)

mmHg 301 grams

Course in the ward:
Plan: Termination of Pregnancy:
Patient underwent
Low Transverse Cesarean Section

For Pre-eclampsia with severe features;

HEELP Syndrome;
Deteriorating maternal Status
 (-) Headache
(-) Epigastric pain
BP ranges: 100-120/70-80mmHg
POST- (+) Grade I bipedal edema

OP
z SGOT
8/25

398
8/26
372
8/27
337
8/28
339
8/29
346
8/30
146
SGPT 463 443 365 386 360
185
LDH 182 357 383
281
z
DISCUSSION
 Twin
P Pregnancy
A
T 1st
Pregnancy
H
O R
P E
H V
Y I
S E
Gr III bipedal
I edema W
O
L
O 140/90 mmHg 300mg
G
Y Epigastric/ Elevated Liver
RUQ pain Enzymes
Headache
HELLP
syndrome
z
 z

HELLP syndrome
a subset of severe preeclampsia/eclampsia with the ff:

 microangiopathic hemolytic anemia

 moderate to severe thrombocytopenia

 disrupted or destroyed erythrocytes on peripheral smear

 abnormal liver function tests presenting with

right upper quadrant/epigastric pain, nausea and
vomiting

CPG, Hypertensive Complications of Pregnancy

How do we
Classify
HELLP
Syndrome?

CPG, Hypertensive Complications of Pregnancy

PATHOPHYSIOLOGY OF HELLP
Am Fam Physician. 1999 Sep 1;60(3):829-836.
 8/25 8/26 8/30
398
z SGOT 372 146
CLINICAL SGPT 463 443 185
LDH 182
281
MANIFESTATIONS

 Develops suddenly in 3rd trimester or immediate postpartum.

 Rapid Progression: 35-50 % decrease in platelets per day and

rise in AST and ALT until 24 – 48 hours postpartum when
levels begin to recover.
 Onset of disease occurs antepartum: 70%, postpartum 30%

 Usually resolves within a week postpartum

CPG, Hypertensive Complications of Pregnancy

 z

CLINICAL
MANIFESTATIONS
 Body malaise (90%)

 Epigastric or right upper quadrant pain (90%)

 Nausea or vomiting (50%)

 Non-specific viral-like symptoms

CPG, Hypertensive Complications of Pregnancy

 z

COMPLICATIONS
OF HELLP
SYNDROME

CPG, Hypertensive Complications of Pregnancy

 z

✔️ Seizure Prophylaxis with
MANAGEMENT magnesium sulfate

✔️ Administration of corticosteroids

HELLP for fetal lung maturity

 Control of hypertension through anti-
SYNDROME
hypertensive medications
is an  Stabilization of maternal condition
INDICATION 
✔️ Serial monitoring of laboratory
For parameters
DELIVERY 
✔️ Close observation of symptoms


✔️ Delivery

CPG, Hypertensive Complications of Pregnancy

 z
❖ Aggressive high dose corticosteroid
therapy has been advocated to improve
MANAGEMENT maternal and neonatal outcome in
HELLP in patient’s with Low platelet
count.
▪ Intravenous dexamethasone 10-12
mg every 12 hours, or
▪ intramuscular betamethasone 10-12
mg every 12 hours, until delivery,
and additional 3 more doses after
deliver
❖ reported improvement in laboratory
values, improvement in blood pressure,
shorter hospital stay and an increased
use of regional anesthesia.

CPG, Hypertensive Complications of Pregnancy

 z
❖ Replacement of clotting factors with frozen
plasma and factor concentrates, and platelet
MANAGEMENT transfusion for counts below 50,000/mm3
should be given as needed
❖ The laboratory abnormalities in HELLP
syndrome typically worsen after delivery and
then begin to resolve by three to four days
postpartum.
❖ Plasmapheresis has been successful in patients
with severe laboratory abnormalities (i.e., a
platelet count of less than 30,000 per mm 3 [30 ×
109 per L] and continued elevation of liver
function values) who have required repeat
transfusions to maintain their hematocrit at 72
hours postpartum.

CPG, Hypertensive Complications of Pregnancy

 z
❖ Replacement of clotting factors with frozen plasma and
MANAGEMENT factor concentrates, and platelet transfusion for counts
below 50,000/mm3 should be given as needed

CPG, Hypertensive Complications of Pregnancy