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HELLP syndrome in Pregnancy

HELLP syndrome can occur in isolation but also occurs as a variant of pre-eclampsia. It was first described as a clinical entity in its own right by a Dr Weinstein in the early 1980s. It is not clearly known why HELLP syndrome develops. The majority of cases are seen during pregnancy but the condition can also occur in the postnatal period.

HELLP syndrome is characterized by three main features which make up its acronym:

Ø Haemolysis: This means the destruction of red blood cells

Ø Elevated Liver enzymes: A blood test will show that the levels of the liver enzymes especially the ‘Tranferases’ will be raised.

Ø Low Platelets: A blood count will show falling platelet count as these are destroyed.

HELLP is a very serious pregnancy complication which can be fatal. Once this is diagnosed, the life of the baby is  in peril and so could the mother’s. Expedited delivery is the only effective answer.

HELLP can occur with no obvious preceding pre-eclampsia. In fact proteinuria, a necessary feature of pre-eclampsia may be absent.

Recognising HELLP Syndrome

Symptoms suggestive of developing HELLP syndrome include

· General malaise

· Headache

· Pain in the right upper abdomen (location of the liver)

· Tingling or ‘pins and needles’ sensation in the extremeties (toes and fingers)

· Nausea and/or vomiting

HELLP syndrome could occur in the absence of most or even all of the above symptoms.

Blood tests will quickly show falling platelet count and elevated liver enzymes as well as a falling hemoglobin level. There will be the telltale features of breakdown of blood cells (hemolysis) as a result of activation of the coagulation cascade and of-course progressive anemia.

HELLP syndrome could lead to what is medically known as consumptive coagulopathy. Basically, this means there is wide-spread coagulation (clotting) taking place in the micro-vasculature and these tiny clots are continually broken down forming a vicious cycle. This is termed Disseminated Intravascular Coagulation (DIC). DIC can lead to an exhaustion of the stores of cloting factors resulting in the exact opposite complication: Uncontrolled hemorrhage.

A test known as D-dimer which detects products of clot breakdown in the blood is sometimes useful in predicting HELLP syndrome complication in patients with pre-eclampsia. It is not a very specific test and hence is not used routinely for this purpose.

Management of HELLP syndrome

Being a potentially very serious condition, it is imperative that once the diagnosis is suspected or confirmed, management should be at a center with the expertise and facilities to deal with this as well as neonatal intensive care.

When this condition develops very remote from ‘Term’ and severe prematurity is inevitable, the baby’s prospects are dire. Close to half of all babies in HELLP syndrome are lost, the main cause being prematurity.

Holding measures once HELLP syndrome has been diagnosed include giving steroids to accelerate fetal lung maturity ready for delivery. Other measures may include blood transfusion, intravenous fluids and anti-hypertensives to control the blood pressure. In the majority of cases, despite the deployment of any of these, delivery will be planned for within 48 hours of diagnosis

If HELLP is complicated by DIC, Fresh frozen Plasma (FFP) may be required to control this very dangerous development.

The bottom line is that delivery of the baby is the only effective cure for HELLP syndrome.

HELLP syndrome complications

HELLP Syndrome can be fatal. Mortality is just over 1%. Other complications that could occur include serious respiratory difficulties (for the mother) in the form of pulmonary edema or Adult Respiratory Distress Syndrome (ARDS), DIC (see above), placental abruption and liver and/or kidney failure.

Role of corticosteroids in HELLP syndrome

There is some evidence that administering corticosteroids such as Dexamethasone might help stabilise the situation in mild or moderate HELLP syndrome to buy vital time for safe delivery of the baby. The aim of these is to arrest the destruction of the platelets and probably the hemolysis to stop progression into full-blown DIC.

The evidence to their effectiveness is not very strong. In any case, these will only be considered where the condition is judged to be mild or moderate and the baby is severely pre-term. There is no place for prevarication when this develops in the third trimester.

Caesarean section with HELLP syndrome

Unless it is absolutely necessary, operative delivery needs to be avoided in this condition. With the risk of DIC and uncontrollable hemorrhage, it is not difficult to see why.

HELLP syndrome recurrence

HELLP syndrome will recur in up to a quarter of all patients in a subsequent pregnancy. When this happens, it tends to at a later stage of pregnancy and generally will be less severe. However, significantly, the risk of pre-eclampsia in a subsequent pregnancy is much higher, at just under 45%.

HELLP syndrome after delivery

HELLP syndrome can occur after delivery. Postnatal HELLP accounts for almost 1 in 12 of all cases. Aggressive management here is aided by the fact that there is no fetal wellbeing to consider.

Contraception after HELLP syndrome

It is perfectly OK to use the oral contraceptive pill after HELLP syndrome once all the liver function tests are back to normal. This is, as long as there are no other contra-indications to the use of the Pill.

Last update: September 30, 2011