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Overview :
The acronym HELLP refers to the combination of Hemolysis,
Elevated Liver enzymes and Low Platelets occurring in a pregnant or postpartum
woman, often associated with pre-eclampsia or eclampsia. It be associated with
significant morbidity and mortality for the mother and infant.
Occurrence:
• The syndrome
may present during the second trimester, third trimester, at term or in the
postpartum period.
• The disorder
may occur at any maternal age and may occur in both primi- and multi-gravida
mothers.
Symptoms:
(1) malaise
(2) epigastric or right upper quadrant pain
(3) nausea or vomiting
(4) viral infection-like symptoms
(5) headache (especially if hypertension present)
Pre-eclampsia or eclampsia is often present but may be
absent.
(1) features of pre-eclampsia: hypertension, proteinuria and
edema
(2) features of eclampsia: features of pre-eclampsia + seizures
Diagnostic features:
(1) hemolysis
(2) elevated liver enzymes
(3) low platelet count
Hemolysis:
• Schistocytes
and other microangiopathic findings are present, indicating intravascular
hemolysis
• If
intravascular hemolysis is sufficiently severe, then the patient will develop
anemia (lower limit of reference range for hemoglobin in young adult women is
11.7 g/dL) an elevated bilirubin, increased serum LDH and decreased serum
haptoglobin.
Elevated liver enzymes:
• The AST (SGOT) increases is greater than the mean of the
reference range + 3SD
• Since most
laboratories use the reference range as the mean +/- 2 SD (to include 95% of
the "normal" population) and the mean = ((lower end) + (upper end)) /
2:
3 SD above the mean =
= (1.25 * (upper end of reference
range)) - (0.25 * (lower end of the reference range))
Low platelet count:
• classically
< 100,000 per µL
• A platelet
count > 100,000 per µL may indicate impending onset if there has been a
significant drop from a higher value.
• A bone marrow biopsy shows increased megakaryocytes.
Differential diagnosis:
(1) DIC (fibrin split products, low fibrinogen)
(2) fatty liver of pregnancy
(3) vasculitis
(4) ITP
(5) TTP/HUS
Diagnosis:
• If all the
features are present, the diagnosis of HELLP may be made.
• The features
in the absence of hemolysis is referred to as the ELLP syndrome.
• Schistocytes
without anemia, rising AST and/or falling platelet count may indicate impending
onset of the syndrome.
Complications:
(1) development
of DIC, which may be associated with occurrence of obstetrical complications
(2) acute renal
failure (increased risk if HELLP onset in postpartum period)
(3) pulmonary
edema (increased risk if HELLP onset in postpartum period)
(4) ARDS
(5) abruptio
placentae
(6)
hepatocellular necrosis
(7) ascites
(8) subcapsular
liver hematoma, which may rupture with intra-abdominal hemorrhage
(9) retinal
detachment
(10) cerebral
edema
Management:
• Early
recognition allows for prompt management.
• The patient
should be placed on strict bed rest, preferably in the left lateral decubitus
position.
• The patient
may benefit from transfer to a larger facility with greater expertise and
resources.
• Fetal
monitoring should be performed. Amniocentesis may be indicated to assess fetal
maturity.
• The fetus
should be delivered if possible. Corticosteroids may be given to enhance fetal
lung development.
• Magnesium
sulfate is given to help prevent convulsions if pre-eclampsia or eclampsia are
present.
•
Antihypertensive medications include a beta-blocker or hydralazine.
• Intravenous
fluid infusion needs to be done carefully. This can help counter the plasma
volume contraction associated with the disease but, if done excessively, may
contribute to peripheral and pulmonary edema. Fluids include crystalloids,
albumin, and fresh frozen plasma (FFP).
• Plasma
exchange may be done in severe cases in the postpartum period.
• Dexamethasone
may be used in severe cases.
• Low-dose
aspirin or heparin are sometimes given.
• Transfusion
of platelets products may be done if the thrombocytopenia becomes severe. It is
important to have excluded DIC if platelet transfusion is being considered.
• If renal
failure develops then dialysis should be considered.
The rate of recurrence in future pregnancies is low (about
3%).
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