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HELLP Syndrome
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HELLP Syndrome
, Hemolysis Elevated Liver Enzymes Low Platelets
See Also
Preeclampsia
Severe Preeclampsia
Definition
Characterized by
Hemolysis
, elevated liver enzymes, and
Low Platelet Count
Variant of
Severe Preeclampsia
Epidemiology
More common in
Multiparous
caucasians
More common after age 25 years
Complicates 20% of
Severe Preeclampsia
cases (but <1% of all pregnancies)
Prevalence
: 6 per 1000 deliveries
Onset after 20 weeks gestation
Preterm <27 weeks: 7% of cases
Preterm <37 weeks: 46-52% of cases
Term (37-41 weeks): 14-18% of cases
Postpartum: 30-33% of cases
Typically within 48 hours of delivery
References
Sibai (1993) Am J Obstet Gynecol 169(4): 1000-6 [PubMed]
Pathophysiology
Microangiopathic
Hemolysis
RBCs fragmented in vessels with damaged endothelium
Fibrin deposition
Vascular deposition contributes to
Hemolysis
Hepatic sinusoid deposition with periportal necrosis
Results in liver enzyme abnormalities
Platelet aggregation
Risk factors
Advanced maternal age
Caucasian
Multiparity
Preeclampsia
HELLP complicates 12% of
Preeclampsia
cases overall (20% of
Severe Preeclampsia
)
However, normal
Blood Pressure
in up to 18% of cases and no
Proteinuria
in 13% of cases
Precautions
HELLP may present in normotensive patients without
Proteinuria
Symptoms
Viral-type prodrome
Malaise
Gastrointestinal symptoms (most common)
Right Upper Quadrant Abdominal Pain
Epigastric Pain
Nausea
or
Vomiting
Other symptoms that are variably present
Headache
Jaundice
Severe Preeclampsia
symptoms may be present
Bleeding may occur if
Coagulopathy
is present
Hematuria
Gastrointestinal Bleeding
Signs
Hypertension
(85% of cases)
Proteinuria
(87% of cases)
Weight gain
Edema
Differential Diagnosis
See
Right Upper Quadrant Abdominal Pain
See
Hemolysis Causes
See
Thrombocytopenia
See
Aminotransferase
Acute Fatty Liver of Pregnancy
Fatty Liver
is associated with more severe liver failure and
Renal Insufficiency
HELLP is associated with
Severe Hypertension
, which is less common in
Fatty Liver
Diagnosis
Hemolysis
Serum Bilirubin
>1.2 mg/dl
Lactate Dehydrogenase
>600 IU/L
Peripheral Smear
with signs of
Hemolysis
Schistocyte
s
Burr Cells
Helmet Cells
Elevated liver enzymes
Liver
transaminases increased (twice normal is a common threshold used)
Low Platelet Count
(
Thrombocytopenia
)
Platelet Count
< 100,000 mm3
Low Platelet Count
by Alternative Classification
Class 3:
Platelet Count
100,000 to 150,000 mm3
Class 2:
Platelet Count
50,000 to 100,000 mm3
Class 1:
Platelet Count
<50,000 mm3
Labs
Consider
Preeclampsia
labs
Complete Blood Count
with platelets
Peripheral Blood Smear
Liver
panel
Aspart
ate transaminase (AST)
Alanine transaminase (ALT)
Serum Bilirubin
Lactate Dehydrogenase
Coagulation Factor
s if platelets <50,000 or bleeding (evaluate for DIC)
Fibrinogen
Fibrin split products
Prothrombin (PT)
Partial Thromboplastin Time
(PTT)
Management
See Severe
Preeclampsia Management
Magnesium Sulfate
Start at admission and continue for 24-48 hours after delivery
Consult maternal fetal medicine
Consider
Dexamethasone
10 mg IV q12 hours
Consider when
Platelet Count
<100,000/mm3
May improve lab abnormalities and delay delivery
Magann (1994) Am J Obstet Gynecol 171:1148-53 [PubMed]
Consider
Blood Product
s
Platelet Transfusion
Platelet Count
<20,000/mm3
Platelet Count
<50,000/mm3 prior to ceserean
Avoid
Regional Anesthesia
(spinal
Anesthesia
) if
Platelet Count
<50,000/mm3
Other
Blood Product
s to consider in active bleeding
Packed Red Blood Cells
for severe blood loss
Anemia
Fresh Frozen Plasma
for coagulation abnormalities
Complications
Hepatic rupture
Acute Renal Failure
Pulmonary Edema
Ascites
Pleural Effusion
Abruptio Placenta
Postpartum Hemorrhage
Disseminated Intravascular Coagulation
(DIC)
Prognosis
Maternal mortality: 1-3%
Infant and fetal mortality: 6-36%
References
Fontaine (2000) in ALSO, B:1-36
Barton (2004) Clin Perinatol 31(4): 807-33 [PubMed]
Leeman (2008) Am Fam Physician 78:93-100 [PubMed]
Leeman (2016) Am Fam Physician 93(2):121-7 [PubMed]
Padden (1999) Am Fam Physician 60:829-39 [PubMed]
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